582
Pathway
Isoleucine Biosynthesis
Isoleucine biosynthesis begins with L-threonine from the threonine biosynthesis pathway. L-threonine interacts with a threonine dehydratase biosynthetic releasing water, a hydrogen ion and (2Z)-2-aminobut-2-enoate. This compound is isomerized into a 2-iminobutanoate which interacts with water and a hydrogen ion spontaneously, resulting in the release of ammonium and 2-ketobutyric acid. This compound reacts with pyruvic acid and hydrogen ion through an acetohydroxybutanoate synthase / acetolactate synthase 2 resulting in carbon dioxide and (S)-2-Aceto-2-hydroxybutanoic acid. The latter compound is reduced by an NADPH driven acetohydroxy acid isomeroreductase releasing NADP and acetohydroxy acid isomeroreductase. The latter compound is dehydrated by a dihydroxy acid dehydratase resulting in 3-methyl-2-oxovaleric acid.This compound reacts in a reversible reaction with L-glutamic acid through a Branched-chain-amino-acid aminotransferase resulting in oxoglutaric acid and L-isoleucine.
L-isoleucine can also be transported into the cytoplasm through two different methods: a branched chain amino acid ABC transporter or a
branched chain amino acid transporter BrnQ
y.
Metabolic
PW000818
Center
PathwayVisualizationContext1105
2325
3150
#000099
PathwayVisualization566
582
Isoleucine Biosynthesis
Isoleucine biosynthesis begins with L-threonine from the threonine biosynthesis pathway. L-threonine interacts with a threonine dehydratase biosynthetic releasing water, a hydrogen ion and (2Z)-2-aminobut-2-enoate. This compound is isomerized into a 2-iminobutanoate which interacts with water and a hydrogen ion spontaneously, resulting in the release of ammonium and 2-ketobutyric acid. This compound reacts with pyruvic acid and hydrogen ion through an acetohydroxybutanoate synthase / acetolactate synthase 2 resulting in carbon dioxide and (S)-2-Aceto-2-hydroxybutanoic acid. The latter compound is reduced by an NADPH driven acetohydroxy acid isomeroreductase releasing NADP and acetohydroxy acid isomeroreductase. The latter compound is dehydrated by a dihydroxy acid dehydratase resulting in 3-methyl-2-oxovaleric acid.This compound reacts in a reversible reaction with L-glutamic acid through a Branched-chain-amino-acid aminotransferase resulting in oxoglutaric acid and L-isoleucine.
L-isoleucine can also be transported into the cytoplasm through two different methods: a branched chain amino acid ABC transporter or a
branched chain amino acid transporter BrnQ
y.
Metabolic
3
109694
581
SubPathway
109192
109
Compound
117
2178
5076220
Bell SC, Turner JM, Collins J, Gray TR: Patterns of microbial threonine catabolism: a survey. Biochem J. 1972 Apr;127(3):77P.
582
Pathway
2179
16743051
Bell SC, Turner JM: Bacterial catabolism of threonine. Threonine degradation initiated by l-threonine hydrolyase (deaminating) in a species of Corynebacterium. Biochem J. 1977 Jun 15;164(3):579-87.
582
Pathway
2180
4573981
Calhoun DH, Rimerman RA, Hatfield GW: Threonine deaminase from Escherichia coli. I. Purification and properties. J Biol Chem. 1973 May 25;248(10):3511-6.
582
Pathway
2181
2675968
Gollop N, Damri B, Barak Z, Chipman DM: Kinetics and mechanism of acetohydroxy acid synthase isozyme III from Escherichia coli. Biochemistry. 1989 Jul 25;28(15):6310-7.
582
Pathway
2182
370104
Grimminger H, Umbarger HE: Acetohydroxy acid synthase I of Escherichia coli: purification and properties. J Bacteriol. 1979 Feb;137(2):846-53.
582
Pathway
2183
355220
Komatsubara S, Murata K, Kisumi M, Chibata I: Threonine degradation by Serratia marcescens. J Bacteriol. 1978 Aug;135(2):318-23.
582
Pathway
2184
378964
Lee-Peng FC, Hermodson MA, Kohlhaw GB: Transaminase B from Escherichia coli: quaternary structure, amino-terminal sequence, substrate specificity, and absence of a separate valine-alpha-ketoglutarate activity. J Bacteriol. 1979 Aug;139(2):339-45.
582
Pathway
2185
13727223
MYERS JW: Dihydroxy acid dehydrase: an enzyme involved in the biosynthesis of isoleucine and valine. J Biol Chem. 1961 May;236:1414-8.
582
Pathway
2186
4562389
Ratzkin B, Arfin S, Umbarger HE: Isoleucine and valine metabolism in Escherichia coli. 18. Induction of acetohydroxy acid isomeroreductase. J Bacteriol. 1972 Oct;112(1):131-41.
582
Pathway
2187
13405870
UMBARGER HE, BROWN B: Threonine deamination in Escherichia coli. II. Evidence for two L-threonine deaminases. J Bacteriol. 1957 Jan;73(1):105-12.
582
Pathway
1
Cell
CL:0000000
5
Hepatocyte
CL:0000182
4
Cardiomyocyte
CL:0000746
3
Neuron
CL:0000540
7
Epithelial Cell
CL:0000066
6
Myocyte
CL:0000187
2
Platelet
CL:0000233
8
Beta cell
CL:0000639
1
Homo sapiens
9606
Eukaryote
Human
3
Escherichia coli
562
Prokaryote
18
Saccharomyces cerevisiae
4932
Eukaryote
Yeast
4
Arabidopsis thaliana
3702
Eukaryote
Thale cress
23
Pseudomonas aeruginosa
287
Prokaryote
12
Mus musculus
10090
Eukaryote
Mouse
5
Bos taurus
9913
Eukaryote
Cattle
17
Rattus norvegicus
10116
Eukaryote
Rat
10
Drosophila melanogaster
7227
Eukaryote
Fruit fly
6
Caenorhabditis elegans
6239
Eukaryote
Roundworm
2
Bacteria
2
Prokaryote
Bacteria
24
Solanum lycopersicum
4081
Eukaryote
Tomato
21
Xenopus laevis
8355
Eukaryote
African clawed frog
25
Escherichia coli (strain K12)
83333
Prokaryote
60
Nitzschia sp.
0001
Eukaryote
Nitzschia4
19
Schizosaccharomyces pombe
4896
Eukaryote
49
Bathymodiolus platifrons
220390
Eukaryote
Deep sea mussel
29
Saccharomyces cerevisiae (strain ATCC 204508 / S288c)
559292
Eukaryote
Baker's yeast
11
Extracellular Space
GO:0005615
1
Cytosol
GO:0005829
31
Periplasmic Space
GO:0005620
35
Chloroplast
GO:0009507
3
Mitochondrial Matrix
GO:0005759
5
Cytoplasm
GO:0005737
2
Mitochondrion
GO:0005739
7
Endoplasmic Reticulum Membrane
GO:0005789
12
Mitochondrial Inner Membrane
GO:0005743
14
Mitochondrial Outer Membrane
GO:0005741
24
Mitochondrial Intermembrane Space
GO:0005758
13
Endoplasmic Reticulum
GO:0005783
4
Peroxisome
GO:0005777
10
Cell Membrane
GO:0005886
36
Membrane
GO:0016020
53
Endoplasmic Reticulum Body
GO:0010168
34
Plant-Type Vacuole
GO:0000325
32
Inner Membrane
GO:0070258
6
Lysosome
GO:0005764
16
Lysosomal Lumen
GO:0043202
18
Melanosome Membrane
GO:0033162
25
Golgi apparatus
GO:0005794
20
Endoplasmic Reticulum Lumen
GO:0005788
21
Synapse
GO:0045202
15
Nucleus
GO:0005634
40
Periplasm
GO:0042597
19
sarcoplasmic reticulum
GO:0016529
27
Peroxisome Membrane
GO:0005778
1
Liver
BTO:0000759
72
9
28
Stomach
BTO:0001307
155
26
8
Blood Vessel
BTO:0001102
74
11
4
Adrenal Medulla
BTO:0000049
71
8
25
Intestine
BTO:0000648
7
Nervous System
BTO:0001484
11
Heart
BTO:0000562
73
10
9
Muscle
BTO:0000887
141
18
24
Brain
BTO:0000142
89
16
5
cardiocyte
BTO:0001539
2
Endothelium
BTO:0000393
18
Pancreas
BTO:0000988
15
11
1
PW_BS000015
2
1
1
1
PW_BS000002
107
31
3
PW_BS000107
108
1
3
PW_BS000108
105
11
3
PW_BS000105
188
1
18
PW_BS000024
187
31
18
PW_BS000024
225
35
4
1
PW_BS000024
318
31
23
PW_BS000024
315
1
23
PW_BS000024
132
1
12
1
PW_BS000132
114
11
12
PW_BS000114
124
1
5
1
PW_BS000124
409
11
5
PW_BS000115
118
1
17
1
PW_BS000118
137
11
17
PW_BS000137
299
1
10
1
PW_BS000024
483
11
10
PW_BS000115
388
1
6
1
PW_BS000112
208
11
6
PW_BS000024
4
3
1
1
PW_BS000004
8
5
1
1
PW_BS000008
31
1
5
1
1
PW_BS000031
3
2
1
1
PW_BS000003
26
1
1
1
5
PW_BS000026
54
1
3
1
5
PW_BS000054
49
7
1
1
PW_BS000049
17
12
1
1
PW_BS000017
22
14
1
1
PW_BS000022
42
24
1
1
PW_BS000042
10
1
7
1
1
PW_BS000010
18
13
1
1
PW_BS000018
70
28
5
1
1
PW_BS000070
103
3
3
1
PW_BS000103
111
5
12
1
PW_BS000111
112
2
12
1
PW_BS000112
100
5
2
1
PW_BS000100
117
1
3
1
PW_BS000117
147
1
24
1
PW_BS000147
155
3
24
1
PW_BS000155
157
2
24
1
PW_BS000157
161
3
18
1
PW_BS000161
159
24
PW_BS000159
166
1
1
PW_BS000166
178
3
21
1
PW_BS000178
152
8
4
PW_BS000152
101
5
3
1
PW_BS000101
163
2
18
1
PW_BS000163
205
5
6
1
PW_BS000024
206
2
6
1
PW_BS000024
219
31
4
PW_BS000024
220
1
4
PW_BS000024
222
3
4
1
PW_BS000024
213
7
18
1
PW_BS000024
210
13
18
1
PW_BS000024
212
1
7
18
1
PW_BS000024
160
1
18
1
PW_BS000160
170
18
PW_BS000170
151
1
4
1
PW_BS000151
226
4
4
1
PW_BS000024
162
12
18
1
PW_BS000162
224
2
4
1
PW_BS000024
195
13
18
PW_BS000024
249
13
4
1
PW_BS000024
164
4
PW_BS000164
281
1
25
1
PW_BS000024
285
10
4
1
PW_BS000024
286
36
4
1
PW_BS000024
287
53
4
1
PW_BS000024
227
34
4
1
PW_BS000024
223
12
4
1
PW_BS000024
294
11
4
1
PW_BS000024
308
10
1
1
PW_BS000024
322
1
23
1
PW_BS000024
312
5
23
1
PW_BS000024
320
11
23
PW_BS000024
293
4
1
PW_BS000024
133
3
12
1
PW_BS000133
134
12
12
1
PW_BS000134
331
7
12
1
PW_BS000028
327
1
1
12
5
PW_BS000028
347
1
3
12
5
PW_BS000028
345
24
12
1
PW_BS000028
130
13
12
1
PW_BS000130
368
3
60
1
PW_BS000028
310
31
2
PW_BS000024
304
1
2
PW_BS000024
119
2
17
1
PW_BS000119
383
7
5
1
PW_BS000100
94
3
PW_BS000094
390
7
6
1
PW_BS000112
398
7
17
1
PW_BS000113
336
1
12
1
PW_BS000028
109
32
3
PW_BS000109
406
3
5
1
PW_BS000115
407
2
5
1
PW_BS000115
122
5
5
1
PW_BS000122
424
1
1
5
5
PW_BS000115
425
1
3
5
5
PW_BS000115
418
24
5
1
PW_BS000115
384
12
5
1
PW_BS000100
125
13
5
1
PW_BS000125
120
3
17
1
PW_BS000120
135
5
17
1
PW_BS000135
459
1
1
17
5
PW_BS000115
460
1
3
17
5
PW_BS000115
454
24
17
1
PW_BS000115
121
12
17
1
PW_BS000121
136
13
17
1
PW_BS000136
479
3
10
1
PW_BS000115
481
2
10
1
PW_BS000115
297
5
10
1
PW_BS000024
495
7
10
1
PW_BS000115
489
24
10
1
PW_BS000115
480
12
10
1
PW_BS000115
300
13
10
1
PW_BS000024
501
3
6
1
PW_BS000115
506
24
6
1
PW_BS000115
391
12
6
1
PW_BS000112
395
13
6
1
PW_BS000113
9
6
1
1
PW_BS000009
5
4
1
1
PW_BS000005
14
10
1
PW_BS000014
13
1
2
1
PW_BS000013
28
1
16
1
1
PW_BS000028
20
4
1
1
1
PW_BS000020
33
18
1
1
PW_BS000033
43
25
1
1
PW_BS000043
24
4
10
1
1
PW_BS000024
60
25
1
PW_BS000060
46
1
1
4
PW_BS000046
29
1
1
1
PW_BS000029
72
5
1
3
PW_BS000072
61
25
1
7
PW_BS000061
36
1
20
1
1
PW_BS000036
37
7
21
1
3
PW_BS000037
93
25
20
1
1
PW_BS000093
27
15
1
PW_BS000027
7
1
1
PW_BS000007
97
1
5
2
1
PW_BS000097
113
6
12
1
PW_BS000113
110
2
3
1
PW_BS000110
123
1
7
5
1
PW_BS000123
126
6
5
1
PW_BS000126
127
1
16
5
1
PW_BS000127
129
1
5
12
1
PW_BS000129
6
1
3
1
PW_BS000006
140
10
3
PW_BS000140
143
1
5
19
1
PW_BS000143
146
5
19
1
PW_BS000146
95
1
7
2
1
PW_BS000095
1
1
PW_BS000001
180
2
21
1
PW_BS000180
207
6
6
1
PW_BS000024
211
10
18
PW_BS000024
214
25
18
1
PW_BS000024
215
6
18
1
PW_BS000024
198
5
18
1
PW_BS000024
216
4
18
1
PW_BS000024
190
11
18
PW_BS000024
277
1
2
18
PW_BS000024
65
11
1
PW_BS000065
290
5
49
1
PW_BS000024
291
6
49
1
PW_BS000024
292
4
49
1
PW_BS000024
298
1
7
10
1
PW_BS000024
301
6
10
1
PW_BS000024
302
1
16
10
1
PW_BS000024
253
5
4
1
PW_BS000024
332
1
7
12
1
PW_BS000028
333
1
2
12
PW_BS000028
115
10
12
PW_BS000115
334
4
12
1
PW_BS000028
337
1
16
12
1
PW_BS000028
341
4
1
12
1
PW_BS000028
343
18
12
1
PW_BS000028
329
14
12
1
PW_BS000028
352
25
12
PW_BS000028
353
25
12
7
PW_BS000028
356
25
12
1
PW_BS000028
360
4
10
12
1
PW_BS000028
370
2
60
1
PW_BS000028
228
36
1
PW_BS000024
232
40
3
PW_BS000024
408
4
5
1
PW_BS000115
412
1
2
5
PW_BS000115
405
10
5
PW_BS000115
415
18
5
1
PW_BS000115
414
1
5
5
1
PW_BS000115
429
1
5
1
PW_BS000115
419
25
5
1
PW_BS000115
434
4
10
5
1
PW_BS000115
382
14
5
1
PW_BS000100
436
25
5
PW_BS000115
374
4
17
1
PW_BS000053
443
6
17
1
PW_BS000115
446
1
2
17
PW_BS000115
376
10
17
PW_BS000053
447
1
7
17
1
PW_BS000115
448
1
16
17
1
PW_BS000115
451
18
17
1
PW_BS000115
450
1
5
17
1
PW_BS000115
464
1
17
1
PW_BS000115
455
25
17
1
PW_BS000115
469
4
10
17
1
PW_BS000115
399
14
17
1
PW_BS000113
471
25
17
PW_BS000115
472
25
17
7
PW_BS000115
482
4
10
1
PW_BS000115
478
10
10
PW_BS000115
487
18
10
1
PW_BS000115
490
25
10
1
PW_BS000115
484
14
10
1
PW_BS000115
502
4
6
1
PW_BS000115
209
10
6
PW_BS000024
504
18
6
1
PW_BS000115
507
25
6
1
PW_BS000115
515
4
10
6
1
PW_BS000115
389
14
6
1
PW_BS000112
513
1
7
6
1
PW_BS000115
50
9
5
1
6
PW_BS000050
156
12
24
1
PW_BS000156
177
1
21
1
PW_BS000177
85
24
10
1
1
PW_BS000085
346
9
5
12
6
PW_BS000028
367
1
60
1
PW_BS000028
423
9
5
5
6
PW_BS000115
458
9
5
17
6
PW_BS000115
35
5
19
1
4
PW_BS000035
71
11
1
3
PW_BS000071
289
2
49
1
PW_BS000024
74
1
3
PW_BS000074
51
8
1
PW_BS000051
326
8
12
PW_BS000028
437
25
5
7
PW_BS000115
416
8
5
PW_BS000115
452
8
17
PW_BS000115
16
2
1
2
PW_BS000016
32
1
15
1
5
PW_BS000032
39
7
1
1
3
PW_BS000039
58
1
14
1
1
PW_BS000058
59
27
1
1
PW_BS000059
66
18
5
1
8
PW_BS000066
23
15
1
1
PW_BS000023
91
8
5
1
1
PW_BS000091
89
2
PW_BS000089
104
14
3
1
PW_BS000104
199
14
18
1
PW_BS000024
217
15
18
PW_BS000024
218
15
18
1
PW_BS000024
350
1
14
12
1
PW_BS000028
128
15
12
1
PW_BS000128
351
15
12
PW_BS000028
335
27
12
1
PW_BS000028
184
1
2
1
PW_BS000024
433
1
14
5
1
PW_BS000115
410
15
5
1
PW_BS000115
422
27
5
1
PW_BS000115
435
15
5
PW_BS000115
468
1
14
17
1
PW_BS000115
444
15
17
1
PW_BS000115
375
27
17
1
PW_BS000053
470
15
17
PW_BS000115
485
15
10
1
PW_BS000115
491
27
10
1
PW_BS000115
499
15
10
PW_BS000115
516
15
6
1
PW_BS000115
508
27
6
1
PW_BS000115
517
15
6
PW_BS000115
47
19
1
4
PW_BS000047
241
5
29
PW_BS000024
257
15
29
1
PW_BS000024
313
23
PW_BS000024
109
L-Threonine
HMDB0000167
Threonine is an essential amino acid in humans. It is abundant in human plasma, particularly in newborns. Severe deficiency of threonine causes neurological dysfunction and lameness in experimental animals. Threonine is an immunostimulant which promotes the growth of thymus gland. It also can probably promote cell immune defense function. This amino acid has been useful in the treatment of genetic spasticity disorders and multiple sclerosis at a dose of 1 gram daily. It is highly concentrated in meat products, cottage cheese and wheat germ. (http://www.dcnutrition.com/AminoAcids/) The threonine content of most of the infant formulas currently on the market is approximately 20% higher than the threonine concentration in human milk. Due to this high threonine content the plasma threonine concentrations are up to twice as high in premature infants fed these formulas than in infants fed human milk. The whey proteins which are used for infant formulas are sweet whey proteins. Sweet whey results from cheese production. Threonine catabolism in mammals appears to be due primarily (70-80%) to the activity of threonine dehydrogenase (EC 1.1.1.103) that oxidizes threonine to 2-amino-3-oxobutyrate, which forms glycine and acetyl CoA, whereas threonine dehydratase (EC 4.2.1.16) that catabolizes threonine into 2-oxobutyrate and ammonia, is significantly less active. Increasing the threonine plasma concentrations leads to accumulation of threonine and glycine in the brain. Such accumulation affects the neurotransmitter balance which may have consequences for the brain development during early postnatal life. Thus, excessive threonine intake during infant feeding should be avoided. (PMID 9853925).
72-19-5
C00188
6288
16857
THR
6051
DB00156
C[C@@H](O)[C@H](N)C(O)=O
C4H9NO3
InChI=1S/C4H9NO3/c1-2(6)3(5)4(7)8/h2-3,6H,5H2,1H3,(H,7,8)/t2-,3+/m1/s1
AYFVYJQAPQTCCC-GBXIJSLDSA-N
(2S,3R)-2-amino-3-hydroxybutanoic acid
119.1192
119.058243159
0.60
3
L-threonine
0
0
FDB011999
Threonin;(2s,3r)-(-)-threonine;(2s,3r)-2-amino-3-hydroxybutyrate;(2s,3r)-2-amino-3-hydroxybutyric acid;(r-(r*,s*))-2-amino-3-hydroxybutanoate;(r-(r*,s*))-2-amino-3-hydroxybutanoic acid;(s)-threonine;2-amino-3-hydroxybutanoate;2-amino-3-hydroxybutanoic acid;2-amino-3-hydroxybutyrate;2-amino-3-hydroxybutyric acid;L-(-)-threonine;L-2-amino-3-hydroxybutyrate;L-2-amino-3-hydroxybutyric acid;L-alpha-amino-beta-hydroxybutyrate;L-alpha-amino-beta-hydroxybutyric acid;Threonine;[r-(r*,s*)]-2-amino-3-hydroxybutanoate;[r-(r*,s*)]-2-amino-3-hydroxybutanoic acid;[r-(r*,s*)]-2-amino-3-hydroxy-butanoate;[r-(r*,s*)]-2-amino-3-hydroxy-butanoic acid;(2s)-threonine;(2s,3r)-2-amino-3-hydroxybutanoic acid;L-threonin;T;Thr;(2s,3r)-2-amino-3-hydroxybutanoate;L-a-amino-b-hydroxybutyrate;L-a-amino-b-hydroxybutyric acid;L-α-amino-β-hydroxybutyrate;L-α-amino-β-hydroxybutyric acid
PW_C000109
Thr
2689
15
2690
2
5644
107
5645
108
5885
105
6908
188
6909
187
8379
225
42414
318
42415
315
79026
132
79038
114
122576
124
122580
409
125148
118
125152
137
126725
299
126734
483
128318
388
128328
208
40034
Hydrogen Ion
HMDB0059597
Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])
C00080
1038
15378
1010
[H+]
H
InChI=1S/p+1
GPRLSGONYQIRFK-UHFFFAOYSA-N
hydron
1.0079
1.007825032
0
hydron
1
0
H+;H(+);Hydrogen cation;Hydron;Proton
PW_C040034
H+
215
4
670
8
753
15
788
31
848
3
1116
2
1463
26
1464
54
2231
49
2780
17
4250
22
4254
42
4547
10
4576
18
4694
70
5241
103
5327
111
5353
112
5626
108
5639
107
5699
100
5720
105
5742
117
5963
147
6037
155
6070
157
6093
161
6130
159
6232
166
6483
178
6601
152
6692
101
6843
188
6910
187
7100
163
7168
205
7191
206
7453
219
7454
220
7472
222
7525
213
7532
210
7558
212
7572
160
7590
170
8195
225
8218
151
8243
226
8413
162
8420
224
9139
195
9155
249
11915
164
12015
281
12181
285
12246
286
12266
287
12521
227
13257
223
13325
294
15330
308
42329
315
42354
318
42401
322
42405
312
42454
320
76912
293
77136
133
77210
134
77372
331
77804
114
77955
132
77990
327
77991
347
78379
345
79929
130
80019
368
80387
310
80388
304
80722
119
93823
124
94823
383
110550
388
112855
94
113280
390
115537
398
115539
118
115856
336
116205
109
119973
406
120193
407
120549
122
120593
409
121170
424
121171
425
122569
418
122615
384
122687
125
122758
120
123183
135
123218
137
123742
459
123743
460
125141
454
125188
121
125273
136
125359
479
125550
481
125730
483
125736
297
125809
299
126517
495
126717
489
126766
480
126823
300
126902
501
127213
208
128308
506
128361
391
128430
395
1420
Water
HMDB0002111
Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).
7732-18-5
C00001
962
15377
937
O
H2O
InChI=1S/H2O/h1H2
XLYOFNOQVPJJNP-UHFFFAOYSA-N
water
18.0153
18.010564686
1
water
0
0
FDB013390
Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;Wasser
PW_C001420
H2O
55
8
94
9
109
5
139
4
151
3
162
14
481
13
526
15
624
28
652
10
691
20
770
33
823
18
838
2
1094
31
1377
49
1465
54
1590
43
2018
24
2532
22
2678
60
2727
46
2778
17
2805
29
3143
70
3164
72
3634
61
4598
36
4727
37
4941
93
5030
27
5156
7
5195
97
5214
100
5227
94
5236
103
5297
105
5319
111
5343
113
5355
112
5402
110
5470
123
5483
125
5492
126
5507
127
5534
130
5537
114
5541
129
5591
135
5608
118
5622
108
5691
6
5759
140
5778
101
5841
143
5853
146
5877
107
5890
95
5910
147
5940
151
6032
155
6059
157
6087
161
6123
163
6133
159
6215
1
6218
166
6477
178
6507
180
6600
152
6713
117
6840
188
6888
160
7162
205
7181
207
7193
206
7211
211
7228
213
7238
214
7243
215
7295
198
7350
216
7388
210
7401
212
7467
222
7492
224
7500
190
7588
170
8201
225
8237
226
8414
162
9265
26
11850
277
11922
164
12011
281
12213
285
12250
286
12264
287
12327
249
12520
227
12632
65
12693
290
12705
291
12715
292
13007
298
13019
300
13025
301
13037
302
13261
223
13327
294
15340
308
42327
315
42695
318
43691
322
76914
293
77019
253
77102
132
77131
133
77215
134
77378
331
77397
332
77471
333
77516
115
77536
334
77628
336
77722
337
77759
341
77816
343
77982
347
78071
329
78235
352
78242
353
78270
356
79113
360
80014
368
80039
370
80591
228
80656
119
93830
383
94794
384
110557
390
110639
391
115844
398
119879
232
119915
122
119963
406
120008
407
120046
408
120113
124
120365
412
120430
405
120438
409
120606
415
120794
414
121158
425
121240
429
121351
121
121381
419
121607
434
122118
382
122384
436
122753
120
122797
374
122804
443
123012
446
123064
376
123072
137
123131
447
123142
136
123162
448
123231
451
123384
450
123730
460
123810
464
123940
455
124165
469
124670
399
124938
471
124945
472
125305
297
125353
479
125386
481
125424
482
125480
299
125682
483
125707
478
125745
487
126054
490
126238
495
126273
484
126764
480
126896
501
126963
502
127017
388
127177
208
127199
209
127227
504
127506
507
127576
515
127836
389
128082
395
128176
513
40939
(2Z)-2-aminobut-2-enoate
(2Z)-2-Aminobut-2-enoate belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon) (2Z)-2-Aminobut-2-enoate is soluble (in water) and a moderately acidic compound (based on its pKa) (2Z)-2-Aminobut-2-enoate may be a unique E.coli metabolite. Conjugate base of 2-aminobut-2-enoic acid.
58739
CC=C(N)C([O-])=O
C4H6NO2
InChI=1S/C4H7NO2/c1-2-3(5)4(6)7/h2H,5H2,1H3,(H,6,7)/p-1
PAWSVPVNIXFKOS-UHFFFAOYSA-M
2-aminobut-2-enoate
100.098
100.040402017
0.45
1
2-aminobut-2-enoate
-1
0
PW_C040939
22A2E
6591
151
8381
225
11923
164
79237
122
80053
285
80144
293
40940
2-iminobutanoate
2-Iminobutanoate belongs to the class of organic compounds known as fatty acids and conjugates. These are aliphatic monocarboxylic acids with a saturated or unsaturated aliphatic tail (with at least 4 Carbon atoms). 2-Iminobutanoate is slightly soluble (in water) and a weakly acidic compound (based on its pKa).
19796801
CCC(=N)C([O-])=O
C4H6NO2
InChI=1S/C4H7NO2/c1-2-3(5)4(6)7/h5H,2H2,1H3,(H,6,7)/p-1
WRBRCYPPGUCRHW-UHFFFAOYSA-M
2-iminobutanoate
100.098
100.040402017
-1.38
1
2-iminobutanoate
-1
0
PW_C040940
2ib
6592
151
8382
225
79238
122
80054
285
22608
Ammonium
HMDB0041827
Ammonium is an important source of nitrogen for many plant species, especially those growing on hypoxic soils. However, it is also toxic to most crop species and is rarely applied as a sole nitrogen source. The ammonium (more obscurely: aminium) cation is a positively charged polyatomic cation with the chemical formula NH4+. It is formed by the protonation of ammonia (NH3). Ammonium is also a general name for positively charged or protonated substituted amines and quaternary ammonium cations (NR4+), where one or more hydrogen atoms are replaced by organic radical groups (indicated by R).
14798-03-9
C01342
16741146
28938
218
[NH4+]
H4N
InChI=1S/H3N/h1H3/p+1
QGZKDVFQNNGYKY-UHFFFAOYSA-O
azanium
18.0385
18.034374133
1
azanium
1
1
Ammonium ion;Ammonia ion;Ammonium;Ammonium chloride;Ammonium(1+);Azanium;Nh4+;[nh4]+;[nh4](+);Nh4(+)
PW_C022608
Ammon
5751
108
5892
95
5969
100
6226
166
8273
151
8367
225
11909
170
12470
249
42627
315
116281
109
3
2-Ketobutyric acid
HMDB0000005
2-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.
600-18-0
C00109
58
30831
2-OXOBUTANOATE
57
DB04553
CCC(=O)C(O)=O
C4H6O3
InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)
TYEYBOSBBBHJIV-UHFFFAOYSA-N
2-oxobutanoic acid
102.0886
102.031694058
-0.11
1
2-oxobutanoic acid
0
-1
FDB003359
2-ketobutanoate;2-ketobutanoic acid;2-ketobutyrate;2-oxo-butanoate;2-oxo-butanoic acid;2-oxo-butyrate;2-oxo-butyric acid;2-oxo-n-butyrate;2-oxo-n-butyric acid;2-oxobutanoate;2-oxobutanoic acid;2-oxobutyrate;2-oxobutyric acid;3-methylpyruvate;3-methylpyruvic acid;Methyl-pyruvate;Methyl-pyruvic acid;Propionyl-formate;Propionyl-formic acid;A-keto-n-butyrate;A-keto-n-butyric acid;A-ketobutyrate;A-ketobutyric acid;A-oxo-n-butyrate;A-oxo-n-butyric acid;A-oxobutyrate;A-oxobutyric acid;Alpha-keto-n-butyrate;Alpha-keto-n-butyric acid;Alpha-ketobutric acid;Alpha-ketobutyrate;Alpha-ketobutyric acid;Alpha-oxo-n-butyrate;Alpha-oxo-n-butyric acid;Alpha-oxobutyrate;Alpha-oxobutyric acid;2-ketobutyric acid;3-methyl pyruvic acid;3-methyl pyruvate;α-ketobutyrate;α-ketobutyric acid;α-oxo-n-butyrate;α-oxo-n-butyric acid
PW_C000003
2KBA
337
8
1868
2
2692
3
8274
151
8383
225
42272
4
78126
132
78163
111
78643
133
79027
112
119922
122
122176
124
122274
406
122577
407
122716
135
124728
118
124829
120
125149
119
125312
297
126333
299
126438
479
126726
481
126858
205
127896
388
128007
501
128319
206
164
Pyruvic acid
HMDB0000243
Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).
127-17-3
C00022
1060
32816
PYRUVATE
1031
DB00119
CC(=O)C(O)=O
C3H4O3
InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)
LCTONWCANYUPML-UHFFFAOYSA-N
2-oxopropanoic acid
88.0621
88.016043994
0.18
1
pyruvic acid
0
-1
FDB008293
2-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeure
PW_C000164
Pyr
17
2
20
4
422
8
1181
3
1449
50
1457
26
5365
103
5405
117
5440
118
5444
120
5566
132
5570
133
5893
95
5920
147
5951
151
6022
155
6067
156
6074
161
6126
160
6383
1
6467
178
6510
177
6532
85
7457
222
7495
220
8200
225
12622
31
15292
249
15349
18
77310
111
77972
346
77978
327
78090
112
80004
368
80042
367
80695
135
112879
94
115683
121
119950
406
120011
124
120175
122
120878
407
121148
423
121154
424
123454
119
123720
458
123726
459
125340
479
125390
299
125534
297
125854
481
126883
501
126931
388
127067
205
127858
206
1316
Carbon dioxide
HMDB0001967
Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.
124-38-9
C00011
280
16526
274
O=C=O
CO2
InChI=1S/CO2/c2-1-3
CURLTUGMZLYLDI-UHFFFAOYSA-N
methanedione
44.0095
43.989829244
0.63
0
carbon dioxide
0
0
DBMET00423
FDB014084
Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744
PW_C001316
CO2
50
8
121
1
204
4
480
13
503
18
640
3
677
31
695
20
806
5
1133
43
1638
49
1745
2
2551
17
3144
70
5283
103
5320
111
5750
108
5771
101
5968
100
6026
155
6078
161
6471
178
6637
107
6922
190
7017
160
7035
163
7061
188
7163
205
7308
198
7333
213
7461
222
7530
210
8215
225
8223
151
9158
249
11849
277
11908
170
12464
226
12688
290
42626
315
43523
318
76994
293
77122
133
77170
132
77470
333
77739
112
77750
129
77763
341
78077
134
78405
356
78427
334
78941
331
79227
130
80008
368
80675
119
80717
135
94836
384
113291
391
115549
121
119954
406
120089
122
120155
407
120364
412
120556
414
120833
419
120922
124
120991
408
121284
125
121505
383
122744
120
123011
446
123190
450
123418
455
123489
118
123556
374
123855
136
124063
398
125344
479
125460
297
125516
481
125824
490
125870
299
125931
482
126280
480
126887
501
127052
206
127277
507
127331
388
127390
502
3002
(S)-2-Aceto-2-hydroxybutanoic acid
HMDB0006900
(S)-2-Aceto-2-hydroxybutanoic acid, also known as (S)-2-hydroxy-2-ethyl-3-oxobutanoate or (S)-2-acetyl-2-hydroxybutyrate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms (S)-2-Aceto-2-hydroxybutanoic acid is soluble (in water) and a weakly acidic compound (based on its pKa) (S)-2-Aceto-2-hydroxybutanoic acid exists in all living organisms, ranging from bacteria to humans. (S)-2-Aceto-2-hydroxybutanoic acid is an intermediate in branched chain amino acid metabolism. It is converted from 2-oxobutanoate or 2-hydoxyethyl ThPP via acetolactate synthase.
C06006
440875
27681
389708
CC[C@](O)(C(C)=O)C(O)=O
C6H10O4
InChI=1S/C6H10O4/c1-3-6(10,4(2)7)5(8)9/h10H,3H2,1-2H3,(H,8,9)/t6-/m0/s1
VUQLHQFKACOHNZ-LURJTMIESA-N
(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid
146.1412
146.057908808
0.25
2
(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid
0
-1
FDB024147
(2s)-2-ethyl-2-hydroxy-3-oxobutanoate;(2s)-2-ethyl-2-hydroxy-3-oxobutanoic acid;(s)-2-aceto-2-hydroxybutanoate;(s)-2-aceto-2-hydroxybutanoic acid;(s)-2-hydroxy-2-ethyl-3-oxobutanoate;(s)-2-hydroxy-2-ethyl-3-oxobutanoic acid;(s)-2-acetyl-2-hydroxybutyric acid;(s)-2-acetyl-2-hydroxybutyrate
PW_C003002
S2A2ha
146
NADPH
HMDB0000221
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.).
53-57-6
C00005
22833512
16474
NADPH
17215925
NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O
C21H30N7O17P3
InChI=1S/C21H30N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1,3-4,7-8,10-11,13-16,20-21,29-31H,2,5-6H2,(H2,23,32)(H,36,37)(H,38,39)(H2,22,24,25)(H2,33,34,35)/t10-,11-,13-,14-,15-,16-,20-,21-/m0/s1
ACFIXJIJDZMPPO-NCHANQSKSA-N
{[(2S,3S,4S,5S)-2-(6-amino-9H-purin-9-yl)-5-[({[({[(2S,3R,4S,5S)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-4-hydroxyoxolan-3-yl]oxy}phosphonic acid
745.4209
745.091102105
-2.14
9
[(2S,3S,4S,5S)-2-(6-aminopurin-9-yl)-5-{[({[(2S,3R,4S,5S)-5-(3-carbamoyl-4H-pyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-4-hydroxyoxolan-3-yl]oxyphosphonic acid
0
-4
FDB021909
2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-b-d-ribofuranosylnicotinamide;2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosylnicotinamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-d-ribofuranosyl-3-pyridinecarboxamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosyl-3-pyridinecarboxamide;Dihydrocodehydrogenase ii;Dihydronicotinamide adenine dinucleotide phosphate;Dihydronicotinamide adenine dinucleotide-p;Dihydrotriphosphopyridine nucleotide reduced;Nadp-reduced;Nadph;Nicotinamide-adenine-dinucleotide-phosphorate;Nicotinamide-adenine-dinucleotide-phosphoric acid;Reduced codehydrase ii;Reduced coenzyme ii;Reduced cozymase ii;Reduced triphosphopyridine nucleotide;Triphosphopyridine nucleotide reduced;B-nadph;B-nicotinamide-adenine-dinucleotide-phosphorate;B-nicotinamide-adenine-dinucleotide-phosphoric acid;Beta-nadph;Beta-nicotinamide-adenine-dinucleotide-phosphorate;Beta-nicotinamide-adenine-dinucleotide-phosphoric acid;Nicotinamide adenine dinucleotide phosphate - reduced
PW_C000146
NADPH
185
8
190
3
778
10
796
5
821
18
837
2
1609
29
1615
49
4687
31
4793
14
4797
14
5310
111
5789
108
5972
147
6128
159
6271
35
6779
117
7068
188
7103
163
7154
205
7205
160
7315
213
7345
210
7559
212
7591
170
8194
225
8219
151
8421
224
11812
198
11893
211
12006
222
12150
164
12245
286
12596
226
12648
249
42343
315
43746
322
76911
293
77166
132
77385
331
77394
332
77460
130
77504
112
77511
115
77623
336
80712
119
113164
94
120105
407
120425
405
120452
122
120616
123
121141
125
121275
429
121402
124
121483
383
123059
376
123086
135
123241
447
123712
136
123846
464
123961
118
124041
398
125472
481
125696
297
126214
299
126529
495
127009
206
127572
388
128101
390
143
NADP
HMDB0000217
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5-phosphate (NMN) coupled by pyrophosphate linkage to the 5-phosphate adenosine 2,5-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.) Hydrogen carrier in biochemical redox systems. In the hexose monophosphoric acid system it is reduced to Dihydrocoenzyme II and reoxidation in the presence of flavoproteins (Dictionary of Organic Compounds).
53-59-8
C00006
5886
18009
NAD(P)
5675
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
C21H29N7O17P3
InChI=1S/C21H28N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1-4,7-8,10-11,13-16,20-21,29-31H,5-6H2,(H7-,22,23,24,25,32,33,34,35,36,37,38,39)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1
XJLXINKUBYWONI-NNYOXOHSSA-O
1-[(2R,3R,4S,5R)-5-[({[({[(2R,3R,4R,5R)-5-(6-amino-9H-purin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
744.4129
744.083277073
-2.27
9
1-[(2R,3R,4S,5R)-5-{[({[(2R,3R,4R,5R)-5-(6-aminopurin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
1
-3
FDB021908
Adenine-nicotinamide dinucleotide phosphate;Codehydrase ii;Codehydrogenase ii;Coenzyme ii;Cozymase ii;Nad phosphate;Nadp;Nadp+;Nicotinamide adenine dinucleotide phosphate;Nicotinamide-adenine dinucleotide phosphate;Tpn;Triphosphopyridine nucleotide;B-nadp;B-nicotinamide adenine dinucleotide phosphate;B-tpn;Beta-nadp;Beta-nicotinamide adenine dinucleotide phosphate;Beta-tpn;Oxidized nicotinamide-adenine dinucleotide phosphate;B-nicotinamide adenine dinucleotide phosphoric acid;Beta-nicotinamide adenine dinucleotide phosphoric acid;β-nicotinamide adenine dinucleotide phosphate;β-nicotinamide adenine dinucleotide phosphoric acid
PW_C000143
NADP
183
8
191
3
768
5
780
10
824
18
839
2
1611
29
1617
49
4685
31
4796
14
4801
14
5308
111
5790
108
6017
147
6132
159
6273
35
6778
117
7069
188
7105
163
7152
205
7206
160
7317
213
7346
210
7562
212
7589
170
8197
225
8220
151
8419
224
11811
198
11897
211
12008
222
12152
164
12249
286
12597
226
12650
249
42344
315
43745
322
76913
293
77164
132
77384
331
77396
332
77461
130
77515
115
77624
336
77814
334
77870
112
80713
119
113165
94
120106
407
120429
405
120450
122
120604
408
120618
123
121142
125
121277
429
121401
124
121485
383
123063
376
123084
135
123229
374
123243
447
123713
136
123848
464
123960
118
124043
398
125473
481
125694
297
125743
482
126215
299
126528
495
127010
206
127225
502
127570
388
128100
390
7698
(R) 2,3-Dihydroxy-3-methylvalerate
HMDB0012140
(R) 2,3-Dihydroxy-methylvalerate is an intermediate in valine, leucine and isoleucine biosynthesis. The pathway of valine biosynthesis is a four-step pathway that shares all of its steps with the parallel pathway of isoleucine biosynthesis. These entwined pathways are part of the superpathway of leucine, valine, and isoleucine biosynthesis, that generates not only isoleucine and valine, but also leucine. (R) 2,3-Dihydroxy-methylvalerate is generated from 3-Hydroxy-3-methyl-2-oxopentanoic acid via the enzyme ketol-acid reductoisomerase (EC 1.1.1.86) then it is converted to (S)-3-methyl-2-oxopentanoic via the dihydroxy-acid dehydratase (EC:4.2.1.9).
562-43-6
C06007
448154
27512
1-KETO-2-METHYLVALERATE
395044
CC[C@@](C)(O)[C@@H](O)C(O)=O
C6H12O4
InChI=1S/C6H12O4/c1-3-6(2,10)4(7)5(8)9/h4,7,10H,3H2,1-2H3,(H,8,9)/t4-,6+/m0/s1
PDGXJDXVGMHUIR-UJURSFKZSA-N
(2R,3R)-2,3-dihydroxy-3-methylpentanoic acid
148.1571
148.073558872
0.52
3
(2R,3R)-2,3-dihydroxy-3-methylpentanoic acid
0
-1
C06007
(2r,3r)-2,3-dihydroxy-3-methylpentanoate;(2r,3r)-2,3-dihydroxy-3-methylpentanoic acid;(r)-2,3-dihydroxy-3-methylpentanoate;(r)-2,3-dihydroxy-3-methylpentanoic acid;(r)-2,3-dihydroxy-3-methylvalerate;1-keto-2-methylvalerate;2,3-dihydroxy-3-methyl-valeric acid;2,3-dihydroxy-3-methylpentanoate;2,3-dihydroxy-3-methylpentanoic acid;2,3-dihydroxy-valerianic acid;4,5-dideoxy-3-c-methyl-pentonic acid;Cid8;Dmv;Alpha,beta-dihydroxy-beta-methylvaleric acid;(2r,3r)-2,3-dihydroxy-3-methylvaleric acid;(2r,3r)-2,3-dihydroxy-3-methylvalerate
PW_C007698
R23D3M
6243
151
373
3-Methyl-2-oxovaleric acid
HMDB0000491
3-Methyl-2-oxovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-methyl-2-oxovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). 3-Methyl-2-oxovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.
1460-34-0
C03465
47
35932
2-KETO-3-METHYL-VALERATE
46
CCC(C)C(=O)C(O)=O
C6H10O3
InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
JVQYSWDUAOAHFM-UHFFFAOYSA-N
3-methyl-2-oxopentanoic acid
130.1418
130.062994186
-1.12
1
3-methyl-2-oxopentanoic acid
0
-1
FDB021447
(3r)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoic acid;(r)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoic acid;(s)-3-methyl-2-oxopentanoate;(s)-3-methyl-2-oxopentanoic acid;2-keto-3-methylvalerate;2-keto-3-methylvaleric acid;2-oxo-3-methyl-n-valerate;2-oxo-3-methyl-n-valeric acid;2-oxo-3-methylpentanoate;2-oxo-3-methylpentanoic acid;2-oxo-3-methylvalerate;2-oxo-3-methylvaleric acid;2-oxoisoleucine;2-oxokolavenate;2-oxokolavenic acid;3-methyl-2-oxo-valerate;3-methyl-2-oxo-valeric acid;3-methyl-2-oxo-pentanoate;3-methyl-2-oxo-pentanoic acid;3-methyl-2-oxopentanoate;3-methyl-2-oxopentanoic acid;3-methyl-2-oxovalerate;3-methyl-2-oxovaleric;A-keto-b-methyl-n-valerate;A-keto-b-methyl-n-valeric acid;A-keto-b-methylvalerate;A-keto-b-methylvaleric acid;A-oxo-b-methyl-n-valerate;A-oxo-b-methyl-n-valeric acid;A-oxo-b-methylvalerate;A-oxo-b-methylvaleric acid;Alpha-keto-beta-methyl-n-valerate;Alpha-keto-beta-methyl-n-valeric acid;Alpha-keto-beta-methylvalerate;Alpha-keto-beta-methylvaleric acid;Alpha-oxo-beta-methyl-n-valerate;Alpha-oxo-beta-methyl-n-valeric acid;Alpha-oxo-beta-methylvalerate;Alpha-oxo-beta-methylvaleric acid;3-ethyl-3-methylpyruvic acid;3-ethyl-3-methylpyruvate;α-keto-β-methyl-n-valerate;α-keto-β-methyl-n-valeric acid;α-keto-β-methylvalerate;α-keto-β-methylvaleric acid;α-oxo-β-methyl-n-valerate;α-oxo-β-methyl-n-valeric acid;α-oxo-β-methylvalerate;α-oxo-β-methylvaleric acid
PW_C000373
3M2Oxva
1725
8
6244
151
79184
111
121547
122
124105
135
95
L-Glutamic acid
HMDB0000148
Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).
56-86-0
C00025
33032
16015
GLT
30572
DB00142
N[C@@H](CCC(O)=O)C(O)=O
C5H9NO4
InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1
WHUUTDBJXJRKMK-VKHMYHEASA-N
(2S)-2-aminopentanedioic acid
147.1293
147.053157781
-0.26
3
L-glutamic acid
0
-1
FDB012535
(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeure
PW_C000095
Glu
16
2
44
3
65
8
119
1
138
4
164
14
969
9
1105
42
1448
50
1456
26
1462
54
5323
111
5344
113
5415
117
5439
118
5565
132
5631
107
5632
108
5859
105
6006
147
6071
157
6191
94
6531
85
6838
187
6844
188
7092
72
7093
71
7165
205
7182
207
7514
224
7518
151
8208
225
8373
220
11792
198
11855
161
12004
222
12621
31
12683
289
12697
290
42348
315
42349
318
42845
320
77020
253
77332
133
77525
112
77971
346
77977
327
77981
347
78291
345
80649
135
120023
124
120040
122
120086
407
120347
406
120692
126
120816
418
121147
423
121153
424
121157
425
122833
119
122997
120
123299
443
123401
454
123719
458
123725
459
123729
460
125401
299
125418
297
125457
481
125667
479
125769
301
125802
489
126941
388
126995
206
127162
501
127257
506
134
Oxoglutaric acid
HMDB0000208
Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).
328-50-7
C00026
51
30915
2-KETOGLUTARATE
50
DB02926
OC(=O)CCC(=O)C(O)=O
C5H6O5
InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)
KPGXRSRHYNQIFN-UHFFFAOYSA-N
2-oxopentanedioic acid
146.0981
146.021523302
-0.44
2
oxoglutarate
0
-2
FDB003361
2-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acid
PW_C000134
AKG
15
2
42
3
141
4
146
8
499
18
673
31
1108
42
1263
5
1447
50
1455
26
1467
54
5375
103
5414
117
5438
118
5564
132
6008
147
6036
155
6069
157
6092
161
6482
178
6530
85
7471
222
7515
224
7519
151
8209
225
8374
220
11863
198
12681
289
77054
253
77135
133
77481
111
77523
112
77746
129
77967
345
77970
346
77976
327
77984
347
78425
334
80018
368
80694
135
113162
94
119972
406
120022
124
120084
407
120174
122
120552
414
120814
418
120989
408
121146
423
121152
424
121160
425
122757
120
122831
119
123186
450
123399
454
123554
374
123718
458
123724
459
123732
460
125357
479
125400
299
125455
481
125533
297
125800
489
125929
482
126900
501
126940
388
126993
206
127066
205
127255
506
127388
502
112
L-Isoleucine
HMDB0000172
Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)-tyrosine, tryptophan and phenylalanine, as well as methionine-are increased in these conditions. Valine, in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. The BCAA are not without side effects. Leucine alone, for example, exacerbates pellagra and can cause psychosis in pellagra patients by increasing excretion of niacin in the urine. Leucine may lower brain serotonin and dopamine. A dose of 3 g of isoleucine added to the niacin regime has cleared leucine-aggravated psychosis in schizophrenic patients. Isoleucine may have potential as an antipsychotic treatment. Leucine is more highly concentrated in foods than other amino acids. A cup of milk contains 800 mg of leucine and only 500 mg of isoleucine and valine. A cup of wheat germ has about 1.6 g of leucine and 1 g of isoleucine and valine. The ratio evens out in eggs and cheese. One egg and an ounce of most cheeses each contain about 400 mg of leucine and 400 mg of valine and isoleucine. The ratio of leucine to other BCAA is greatest in pork, where leucine is 7 to 8 g and the other BCAA together are only 3 to 4 g. (http://www.dcnutrition.com).
73-32-5
C00407
6306
17191
ILE
6067
DB00167
CC[C@H](C)[C@H](N)C(O)=O
C6H13NO2
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
AGPKZVBTJJNPAG-WHFBIAKZSA-N
(2S,3S)-2-amino-3-methylpentanoic acid
131.1729
131.094628665
-0.06
2
L-isoleucine
0
0
FDB012397
(2s,3s)-2-amino-3-methylpentanoate;(2s,3s)-2-amino-3-methylpentanoic acid;(2s,3s)-2-amino-3-methyl-pentanoate;(2s,3s)-2-amino-3-methyl-pentanoic acid;(2s,3s)-a-amino-b-methyl-n-valerate;(2s,3s)-a-amino-b-methyl-n-valeric acid;(2s,3s)-a-amino-b-methylvalerate;(2s,3s)-a-amino-b-methylvaleric acid;(2s,3s)-alph-amino-beta-methylvalerate;(2s,3s)-alph-amino-beta-methylvaleric acid;(2s,3s)-alpha-amino-beta-merthyl-n-valerate;(2s,3s)-alpha-amino-beta-merthyl-n-valeric acid;(2s,3s)-alpha-amino-beta-merthylvalerate;(2s,3s)-alpha-amino-beta-merthylvaleric acid;(2s,3s)-alpha-amino-beta-methyl-n-valerate;(2s,3s)-alpha-amino-beta-methyl-n-valeric acid;(2s,3s)-alpha-amino-beta-methylvalerate;(2s,3s)-alpha-amino-beta-methylvaleric acid;(s)-isoleucine;(s,s)-isoleucine;2-amino-3-methylpentanoate;2-amino-3-methylpentanoic acid;2-amino-3-methylvalerate;2-amino-3-methylvaleric acid;2s,3s-isoleucine;Erythro-l-isoleucine;Ile;Iso-leucine;Isoleucine;L-(+)-isoleucine;L-ile;[s-(r*,r*)]-2-amino-3-methylpentanoate;[s-(r*,r*)]-2-amino-3-methylpentanoic acid;Alpha-amino-beta-methylvaleric acid;I;A-amino-b-methylvalerate;A-amino-b-methylvaleric acid;Alpha-amino-beta-methylvalerate;α-amino-β-methylvalerate;α-amino-β-methylvaleric acid
PW_C000112
Ile
1724
8
5656
107
5657
108
7147
187
7148
188
42542
315
42562
318
79183
111
121546
122
124104
135
458
Sodium
HMDB0000588
Sodium ions are necessary for regulation of blood and body fluids, transmission of nerve impulses, heart activity, and certain metabolic functions. Physiologically, it exists as an ion in the body. Sodium is needed by animals, which maintain high concentrations in their blood and extracellular fluids, but the ion is not needed by plants. The human requirement for sodium in the diet is less than 500 mg per day, which is typically less than a tenth as much as many diets "seasoned to taste." Most people consume far more sodium than is physiologically needed. For certain people with salt-sensitive blood pressure, this extra intake may cause a negative effect on health.
17341-25-2
C01330
923
29101
899
[Na+]
Na
InChI=1S/Na/q+1
FKNQFGJONOIPTF-UHFFFAOYSA-N
sodium(1+) ion
22.9898
22.989769675
0
sodium(1+) ion
1
1
FDB003523
Sodium;Sodium ion;Na(+);Na+
PW_C000458
Na+
2093
15
2094
2
2724
60
2725
61
3157
71
3158
72
3185
74
3615
51
4607
8
5860
105
5861
107
5862
108
7149
187
7150
188
42337
318
42338
315
78239
353
78243
326
78249
352
79047
132
122389
436
122390
437
122635
416
122636
124
124942
472
124946
452
124960
471
125209
118
43013
2-Keto-3-methyl-valerate
(3S)-3-Methyl-2-oxopentanoate, also known as (S)-2-oxo-3-methylpentanoate or α-keto-methylvalerate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms (3S)-3-Methyl-2-oxopentanoate is soluble (in water) and a weakly acidic compound (based on its pKa) (3S)-3-Methyl-2-oxopentanoate can be biosynthesized from valerate..
6857401
35146
CC[C@H](C)C(=O)C([O-])=O
C6H9O3
InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)/p-1/t4-/m0/s1
JVQYSWDUAOAHFM-BYPYZUCNSA-M
(3S)-3-methyl-2-oxopentanoate
129.1339
129.055169154
-1.07
0
(S)-3-methyl-2-oxopentanoate
-1
-1
α-keto-β-methyl-valerate;-keto-methylvalerate;(3<i>s</i>)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoate;(3r)-3-methyl-2-oxopentanoic acid;(3s)-3-methyl-2-oxopentanoate;(3s)-3-methyl-2-oxopentanoic acid;(<i>s</i>)-2-oxo-3-methylpentanoate;(<i>s</i>)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoate;(r)-3-methyl-2-oxopentanoic acid;(s)-2-oxo-3-methylpentanoate;(s)-2-oxo-3-methylpentanoic acid;(s)-3-methyl-2-oxopentanoate;(s)-3-methyl-2-oxopentanoic acid;(s)-3-methyl-2-oxovalerate;(s)-3-methyl-2-oxovaleric acid;2-keto-3-methyl-valerate;2-keto-3-methyl-valeric acid;2-keto-3-methylvalerate;2-keto-3-methylvaleric acid;2-oxo-3-methyl-n-valerate;2-oxo-3-methyl-n-valeric acid;2-oxo-3-methylpentanoate;2-oxo-3-methylpentanoic acid;2-oxo-3-methylvalerate;2-oxo-3-methylvaleric acid;2-oxoisoleucine;2-oxokolavenate;2-oxokolavenic acid;3-methyl-2-oxo-pentanoate;3-methyl-2-oxo-pentanoic acid;3-methyl-2-oxo-valerate;3-methyl-2-oxo-valeric acid;3-methyl-2-oxopentanoate;3-methyl-2-oxopentanoic acid;3-methyl-2-oxovalerate;3-methyl-2-oxovaleric;3-methyl-2-oxovaleric acid;A-keto-b-methyl-n-valerate;A-keto-b-methyl-n-valeric acid;A-keto-b-methylvalerate;A-keto-b-methylvaleric acid;A-oxo-b-methyl-n-valerate;A-oxo-b-methyl-n-valeric acid;A-oxo-b-methylvalerate;A-oxo-b-methylvaleric acid;Alpha-keto-beta-methyl-n-valerate;Alpha-keto-beta-methyl-n-valeric acid;Alpha-keto-beta-methyl-valerate;Alpha-keto-beta-methyl-valeric acid;Alpha-keto-beta-methylvalerate;Alpha-keto-beta-methylvaleric acid;Alpha-keto-methylvalerate;Alpha-keto-methylvaleric acid;Alpha-oxo-beta-methyl-n-valerate;Alpha-oxo-beta-methyl-n-valeric acid;Alpha-oxo-beta-methylvalerate;Alpha-oxo-beta-methylvaleric acid
PW_C043013
2K3MVal
414
Adenosine triphosphate
HMDB0000538
Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).
56-65-5
C00002
5957
15422
ATP
5742
DB00171
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
C10H16N5O13P3
InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1
ZKHQWZAMYRWXGA-KQYNXXCUSA-N
({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid
507.181
506.995745159
-2.05
7
adenosine triphosphate
0
-3
FDB021813
5'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acid
PW_C000414
ATP
9
2
21
4
60
8
266
16
414
22
478
13
733
32
799
5
934
39
976
3
2105
18
2112
10
2146
49
2156
14
2160
58
2405
59
2434
27
2726
46
2812
29
3029
66
3163
72
3616
61
3617
51
4399
23
4474
31
4768
91
4864
54
5032
89
5035
26
5155
7
5205
97
5215
100
5250
104
5291
101
5313
111
5346
112
5390
103
5406
117
5430
118
5443
120
5542
129
5556
132
5569
133
5603
135
5621
108
5846
143
5854
146
5876
107
5897
147
5924
151
6048
155
6109
161
6230
166
6493
178
6839
188
6870
160
6976
199
7157
205
7184
206
7209
210
7225
213
7229
211
7298
198
7302
216
7390
217
7408
218
7432
163
7481
222
7499
190
8186
225
11847
277
11903
170
12010
281
12039
164
12178
285
12578
226
12691
290
13264
223
15327
308
42326
315
42621
322
42694
318
77028
253
77218
134
77233
329
77468
333
77632
336
78037
332
78041
350
78168
128
78214
351
78240
353
78411
335
78494
115
78850
130
78865
331
78919
334
80028
368
80046
184
80674
119
85629
1
94826
124
113234
94
113282
388
116280
109
119914
122
119992
406
120154
407
120245
382
120362
412
121246
429
121392
123
121397
433
121471
408
121974
410
122065
125
122079
383
122083
405
122402
422
122444
435
122919
399
123009
446
123816
464
123951
447
123956
468
124029
374
124527
444
124616
136
124630
398
124634
376
124943
472
124972
375
125011
470
125304
297
125371
479
125392
299
125515
481
125595
484
126123
485
126220
300
126234
495
126240
478
126547
491
126596
499
126913
501
127123
389
127731
516
127781
395
127796
390
127801
209
128119
508
128167
517
1104
Phosphate
HMDB0001429
Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.
14265-44-2
C00009
1061
18367
CPD-8587
1032
[O-]P([O-])([O-])=O
O4P
InChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)/p-3
NBIIXXVUZAFLBC-UHFFFAOYSA-K
phosphoric acid
94.9714
94.95342
3
phosphoric acid
0
-2
DBMET00532
FDB022617
Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ion
PW_C001104
Pi
24
4
84
8
814
5
818
18
831
2
980
3
1763
14
1767
49
2500
10
2729
47
2737
46
3129
29
3166
72
3636
61
3851
23
4249
22
4475
31
5031
27
5158
7
5207
97
5216
100
5317
111
5351
112
5381
103
5447
120
5543
129
5573
133
5605
135
5625
108
5693
6
5848
143
5855
146
5911
147
5941
151
6040
155
6100
161
6294
107
6487
178
6691
101
6714
117
6842
188
6889
160
7161
205
7189
206
7212
211
7306
198
7389
210
7402
212
7436
163
7475
222
8196
225
8258
227
10118
241
10134
257
11748
132
11761
115
11773
213
11904
170
11927
164
12014
281
12728
290
13263
223
34819
17
42255
304
42350
315
42435
318
43692
322
77018
253
77194
293
77217
134
77940
336
77966
130
78048
332
78057
329
78245
353
78669
331
80022
368
89279
308
93831
383
94796
384
110558
390
110640
391
113235
94
115845
398
116206
109
119982
406
120069
122
120699
407
121057
124
121216
125
121268
429
121352
121
121409
123
121423
382
121852
405
123304
119
123621
118
123786
136
123838
464
123968
447
123981
399
124405
376
124948
472
125362
479
125446
297
125774
481
125954
299
126221
478
126594
300
126604
298
126723
484
126904
501
127413
388
127783
209
128166
395
128177
513
128315
389
1034
Adenosine diphosphate
HMDB0001341
Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.
58-64-0
C00008
6022
16761
ADP
5800
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
C10H15N5O10P2
InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1
XTWYTFMLZFPYCI-KQYNXXCUSA-N
[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid
427.2011
427.029414749
-2.12
6
adenosine-diphosphate
0
-2
FDB021817
Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acid
PW_C001034
ADP
23
4
134
8
415
22
482
13
801
5
963
15
978
3
1061
14
1518
2
1901
49
2104
18
2113
10
2161
58
2408
59
2435
27
2728
47
2736
46
2855
29
3165
72
3635
61
4400
23
4476
31
4770
91
5036
26
5157
7
5208
97
5217
100
5315
111
5349
112
5392
103
5446
120
5544
129
5572
133
5624
108
5741
117
5764
101
5849
143
5856
146
5878
107
5899
147
5926
151
6050
155
6111
161
6231
166
6495
178
6700
94
6841
188
6872
160
7159
205
7187
206
7208
210
7226
213
7231
211
7300
198
7303
216
7391
217
7410
218
7433
163
7483
222
8187
225
11851
277
11905
170
12013
281
12180
285
13262
223
15329
308
42328
315
42398
313
42622
322
42696
318
77029
253
77087
132
77216
134
77306
329
77472
333
77663
336
78039
332
78043
350
78170
128
78215
351
78244
353
78414
335
78495
115
78705
331
78849
130
78920
334
80030
368
80622
118
80651
135
80676
119
94827
124
113283
388
116204
109
119944
122
119994
406
120156
407
120318
382
120366
412
121248
429
121394
123
121399
433
121472
408
121899
383
121976
410
122064
125
122085
405
122405
422
122445
435
122973
399
123013
446
123818
464
123953
447
123958
468
124030
374
124452
398
124529
444
124615
136
124636
376
124947
472
124975
375
125012
470
125334
297
125373
479
125492
299
125517
481
125645
484
126125
485
126219
300
126235
495
126242
478
126550
491
126597
499
126915
501
127733
516
127780
395
127797
390
127803
209
128122
508
128168
517
128313
389
5875
Threonine dehydratase biosynthetic
P04968
Involved in catalytic activity. Catalyzes the formation of alpha-ketobutyrate from threonine in a two-step reaction. The first step is a dehydration of threonine, followed by rehydration and liberation of ammonia. Deaminates L-threonine, but also L-serine to a lesser extent.
ilvA
b3772
AP009048
3
4.3.1.19
7615
Acetolactate synthase isozyme 2 large subunit
P00892
Amino acid transport and metabolism. Catalyzes the first step in the biosynthesis of branched-chain amino acids.
ilvG
M10313
3
2.2.1.6
6221
Acetolactate synthase isozyme 2 small subunit
P0ADG1
Involved in acetolactate synthase activity. 2 pyruvate = 2-acetolactate + CO(2).
ilvM
b3769
AP009048
3
2.2.1.6
5937
Acetolactate synthase isozyme 1 large subunit
P08142
Involved in magnesium ion binding. 2 pyruvate = 2-acetolactate + CO(2).
ilvB
b3671
AP009048
3
2.2.1.6
6220
Acetolactate synthase isozyme 1 small subunit
P0ADF8
Involved in amino acid binding. 2 pyruvate = 2-acetolactate + CO(2).
ilvN
b3670
AP009048
3
2.2.1.6
5839
Acetolactate synthase isozyme 3 large subunit
P00893
Involved in magnesium ion binding. 2 pyruvate = 2-acetolactate + CO(2).
ilvI
b0077
AP009048
3
2.2.1.6
5840
Acetolactate synthase isozyme 3 small subunit
P00894
Involved in acetolactate synthase activity. 2 pyruvate = 2-acetolactate + CO(2).
ilvH
b0078
AP009048
3
2.2.1.6
5890
Ketol-acid reductoisomerase
P05793
Involved in oxidation-reduction process. (R)-2,3-dihydroxy-3-methylbutanoate + NADP(+) = (S)-2-hydroxy-2-methyl-3-oxobutanoate + NADPH.
ilvC
b3774
AP009048
3
1.1.1.86
5793
108
5889
Dihydroxy-acid dehydratase
P05791
Involved in metabolic process. 2,3-dihydroxy-3-methylbutanoate = 3-methyl-2- oxobutanoate + H(2)O.
ilvD
b3771
AP009048
3
4.2.1.9
7307
Branched-chain-amino-acid aminotransferase
P0AB80
Involved in catalytic activity. Acts on leucine, isoleucine and valine.
ilvE
b3770
AP009048
3
2.6.1.42
7435
Branched-chain amino acid transport system 2 carrier protein
P0AD99
Involved in branched-chain aliphatic amino acid transmembrane transporter activity. Component of the LIV-II transport system for branched- chain amino acids. This LIV-II transport system may be H(+)- coupled.
brnQ
b0401
AP009048
3
8708
109
7389
High-affinity branched-chain amino acid transport ATP-binding protein livF
P22731
Involved in nucleotide binding. Component of the leucine-specific transport system.
livF
b3454
AP009048
3
5648
109
135813
117
7350
High-affinity branched-chain amino acid transport ATP-binding protein livG
P0A9S7
Involved in nucleotide binding. Component of the leucine-specific transport system.
livG
b3455
AP009048
3
5649
109
135814
117
7051
High-affinity branched-chain amino acid transport system permease protein livH
P0AEX7
Part of the binding-protein-dependent transport system for branched-chain amino acids. Probably responsible for the translocation of the substrates across the membrane.
livH
b3457
AP009048
3
5650
109
135815
117
7096
High-affinity branched-chain amino acid transport system permease protein livM
P22729
Part of the binding-protein-dependent transport system for branched-chain amino acids. Probably responsible for the translocation of the substrates across the membrane.
livM
b3456
AP009048
3
5651
109
135816
117
7377
Leu/Ile/Val-binding protein
P0AD96
Involved in amino acid transport. This protein is a component of the leucine, isoleucine, valine, (threonine) transport system, which is one of the two periplasmic binding protein-dependent transport systems of the high-affinity transport of the branched-chain amino acids.
livJ
b3460
AP009048
3
5652
109
135817
117
1529
threonine deaminase
3
PW_P001529
1798
5875
4
1530
acetohydroxybutanoate synthase / acetolactate synthase
3
PW_P001530
1799
7615
1800
6221
1531
acetohydroxybutanoate synthase / acetolactate synthase 2
3
PW_P001531
1801
5937
2
1802
6220
2
1532
acetolactate synthase / acetohydroxybutanoate synthase 3
3
PW_P001532
1803
5839
2
1804
5840
2
1498
acetohydroxy acid isomeroreductase
3
PW_P001498
1763
5890
4
1499
dihydroxy acid dehydratase
3
PW_P001499
1764
5889
2
1495
branched-chain amino-acid aminotransferase
3
PW_P001495
1758
7307
6
3780
branched chain amino acid transporter BrnQ
3
PW_P003780
10694
7435
1443
branched chain amino acid ABC transporter
3
PW_P001443
1685
7389
1686
7350
1687
7051
1688
7096
1689
7377
2922
false
PW_R002922
Right
11613
109
1
Compound
false
11614
40034
1
Compound
false
11615
1420
1
Compound
false
11616
40939
1
Compound
false
2888
1529
4.3.1.19
2923
true
PW_R002923
Right
11617
40939
1
Compound
false
11618
40940
1
Compound
false
2924
true
PW_R002924
Right
11619
40940
1
Compound
false
11620
1420
1
Compound
false
11621
40034
1
Compound
false
11622
22608
1
Compound
false
11623
3
1
Compound
false
2925
false
PW_R002925
Right
11624
3
1
Compound
false
11625
40034
1
Compound
false
11626
164
1
Compound
false
11627
1316
1
Compound
false
11628
3002
1
Compound
false
2889
1530
2.2.1.6
2890
1531
2.2.1.6
2891
1532
2.2.1.6
2926
false
PW_R002926
Right
11629
3002
1
Compound
false
11630
146
1
Compound
false
11631
40034
1
Compound
false
11632
143
1
Compound
false
11633
7698
1
Compound
false
2892
1498
2927
false
PW_R002927
Right
11634
7698
1
Compound
false
11635
1420
1
Compound
false
11636
373
1
Compound
false
2893
1499
2928
false
PW_R002928
Right
11637
373
1
Compound
false
11638
95
1
Compound
false
11639
134
1
Compound
false
11640
112
1
Compound
false
2894
1495
5890
true
PW_R005890
Both
24027
373
1
Compound
false
24028
43013
1
Compound
false
447
PW_RCT000447
Right
2472
447
112
Compound
1
107
2473
447
414
Compound
1
117
2474
447
1420
Compound
1
117
2475
447
112
Compound
1
117
2476
447
1034
Compound
1
117
2477
447
1104
Compound
1
117
2478
447
40034
Compound
1
117
447
445
PW_T000445
550
112
1
Compound
107
108
Right
551
458
1
Compound
107
108
Right
382
3780
2015-06-16T11:54:24-06:00
2015-06-16T11:54:24-06:00
109
15703
109
3
false
750
617
10
regular
100
100
15704
40034
55
false
1101
725
10
regular
78
78
15705
1420
49
false
1101
548
10
regular
78
78
15706
40939
3
false
1310
611
10
regular
100
120
15707
40940
3
false
1535
616
10
regular
100
110
15708
1420
49
false
1681
550
10
regular
78
78
15709
40034
55
false
1711
723
10
regular
78
78
15710
22608
3
false
1895
522
10
regular
100
100
15711
3
3
false
2080
615
10
regular
100
110
15712
40034
55
false
2200
721
10
regular
78
78
15713
164
3
false
2190
525
10
regular
100
100
15714
1316
52
false
2561
761
10
regular
78
78
15715
3002
3
false
2439
875
10
regular
100
130
15716
146
62
false
2394
1147
10
regular
50
30
15717
40034
55
false
2540
1082
10
regular
78
78
15718
143
61
false
2369
1373
10
regular
50
30
15719
7698
3
false
2439
1458
10
regular
100
120
15720
1420
49
false
2000
1574
10
regular
78
78
15721
373
3
false
1603
1453
10
regular
100
120
15722
95
3
false
1318
1558
10
regular
100
110
15723
134
3
false
968
1563
10
regular
100
110
15724
112
117
3
false
753
1463
10
regular
100
100
22344
458
107
58
false
670
1940
10
regular
78
78
22345
458
108
58
false
685
1725
10
regular
78
78
22346
112
107
3
false
195
1276
10
regular
100
100
69385
43013
3
false
1603
1693
10
regular
100
120
2678336
112
107
3
false
755
2029
10
regular
100
100
2678344
414
117
42
false
515
1448
10
regular
50
30
2678345
1420
117
49
false
511
1508
10
regular
78
78
2678347
1104
117
46
false
528
1041
10
regular
44
43
2678349
40034
117
55
false
531
874
10
regular
78
78
2678374
1034
117
43
false
530
984
10
regular
50
30
7858
5875
8
false
915
625
8
subunit
regular
140
85
7859
7615
2
false
2305
640
8
subunit
regular
150
70
7860
6221
2
false
2455
510
8
subunit
regular
150
70
7861
5937
6
false
2425
565
8
subunit
regular
160
80
7862
6220
6
false
2410
630
8
subunit
regular
160
80
7863
5839
6
false
2315
575
8
subunit
regular
160
80
7864
5840
6
false
2335
520
8
subunit
regular
160
80
7865
5890
8
false
2419
1228
8
subunit
regular
140
85
7866
5889
6
false
2109
1473
8
subunit
regular
160
80
7867
7307
17
false
1113
1473
8
subunit
regular
180
85
10716
7435
109
76
false
729
1844
8
subunit
regular
150
70
947985
7389
109
76
false
345
1089
8
subunit
regular
150
70
947986
7350
109
76
false
345
1154
8
subunit
regular
150
70
947987
7051
109
76
false
345
1219
8
subunit
regular
150
70
947988
7096
109
76
false
345
1289
8
subunit
regular
150
70
947989
7377
109
76
false
345
1359
8
subunit
regular
150
70
6316
1529
566
7759
7858
6317
1530
566
7760
7859
7761
7860
6318
1531
566
7762
7861
7763
7862
6319
1532
566
7764
7863
7765
7864
6320
1498
566
7766
7865
6321
1499
566
7767
7866
6322
1495
566
7768
7867
8388
3780
566
10538
10716
802864
1443
566
109
943865
947985
943866
947986
943867
947987
943868
947988
943869
947989
23927
M850 667 C880 665 885 665 915 665
5
false
18
23928
M1140 725 C1138 658 1085 665 1055 665
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23929
M1140 626 C1140 669 1085 665 1055 665
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23930
M1310 671 C1280 671 1085 665 1055 665
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23931
M1410 671 C1432 671 1432 671 1462 671
5
false
18
23932
M1535 671 C1510 671 1485 671.5 1453 671.5
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23933
M1635 671 C1665 671 1952 671 1982 671
5
false
18
23934
M1720 628 C1737 663 1806 671 1864 671
5
false
18
23935
M1750 723 C1759 672 1815 671 1845 671
5
false
18
23936
M1945 622 C1939 664 1896 671 1866 671
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23937
M2080 670 C2050 670 1999 671 1969 671
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23938
M2180 670 C2209 671 2389 670 2410 670
5
false
18
23939
M2239 721 C2238 682 2367 671 2410 670
5
false
18
23940
M2240 625 C2242 661 2355 670 2410 670
5
false
18
23941
M2561 800 C2494 798 2490 740 2490 710
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23942
M2489 875 C2489 845 2490 740 2490 710
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23943
M2489 1005 C2489 1049 2490 1168 2489 1228
5
false
18
23944
M2448 1162 C2466 1162 2489 1168 2489 1228
5
false
18
23945
M2540 1121 C2508 1119 2489 1170 2489 1228
5
false
18
23946
M2423 1388 C2480 1386 2487 1343 2489 1313
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23947
M2489 1458 C2487 1407 2490 1341 2489 1313
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23948
M2439 1518 C2395 1517 2304 1512 2269 1513
5
false
18
23949
M2039 1574 C2039 1540 2069 1513 2109 1513
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23950
M1703 1513 C1745 1512 2084 1512 2109 1513
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23951
M1603 1513 C1573 1513 1323 1513 1293 1513
5
false
18
true
M 894.0096189432334 1720.5 L 907 1713 L 894.0096189432334 1705.5
false
23952
M1368 1558 C1369 1509 1323 1513 1293 1513
5
false
18
true
M 810.0315748257511 1626.2497907707957 L 803 1613 L 795.0411320105379 1625.7144178131095
false
23953
M1018 1563 C1019 1507 1083 1513 1113 1513
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
23954
M853 1513 C883 1513 1083 1513 1113 1513
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
98740
M1653 1573 C1653 1621 1655 1612 1655 1641
5
false
18
true
M 25.946855044164835 1064.9282229629628 L 11 1063.6666666666667 L 17.380887721185843 1077.2418009897451
false
98741
M1653 1693 C1653 1645 1652 1632 1652 1592
5
false
18
true
M 25.946855044164835 1064.9282229629628 L 11 1063.6666666666667 L 17.380887721185843 1077.2418009897451
false
3657518
M803 1563 C803 1613 804 1820 804 1844
83
false
18
true
M 654.6921397433352 1781.4244279451927 L 648 1768 L 639.720174239857 1780.5077769960003
false
3657519
M805 2029 C803 1989 806 1962 804 1914
83
false
18
false
false
3657520
M748 1979 C774 1979 804 1944 804 1914
83
false
18
3657521
M763 1764 C796 1767 804 1814 804 1844
83
false
18
true
M 25.946855044164835 1635.261556296296 L 11 1634 L 17.380887721185843 1647.5751343230784
false
3657582
M750 667 C720 667 720 667 675 667
5
false
18
3657641
M295 1326 C325 1326 315 1326 345 1324
5
false
18
3657642
M515 1463 C466 1464 423 1458 420 1429
5
false
18
3657643
M511 1547 C479 1549 422 1491 420 1429
5
false
18
3657644
M803 1463 C803 1407 802 1366 802 1327 C746 1328 604 1323 495 1324
5
false
18
true
M 25.946855044164835 1440.261556296296 L 11 1439 L 17.380887721185843 1452.5751343230784
false
3657645
M530 999 C466 993 431 1021 420 1089
5
false
18
true
M 25.946855044164835 1440.261556296296 L 11 1439 L 17.380887721185843 1452.5751343230784
false
3657646
M528 1062.5 C498 1062.5 445 1054 420 1089
5
false
18
true
M 25.946855044164835 1440.261556296296 L 11 1439 L 17.380887721185843 1452.5751343230784
false
3657647
M531 913 C450 912 425 1015 420 1089
5
false
18
true
M 25.946855044164835 1440.261556296296 L 11 1439 L 17.380887721185843 1452.5751343230784
false
4164
566
2922
14414
15703
23927
Left
14415
15704
23928
Right
14416
15705
23929
Right
14417
15706
23930
Right
4334
2888
6316
4165
566
2923
14418
15706
23931
Left
14419
15707
23932
Right
4166
566
2924
14420
15707
23933
Left
14421
15708
23934
Left
14422
15709
23935
Left
14423
15710
23936
Right
14424
15711
23937
Right
4167
566
2925
14425
15711
23938
Left
14426
15712
23939
Left
14427
15713
23940
Left
14428
15714
23941
Right
14429
15715
23942
Right
4335
2889
6317
4336
2890
6318
4337
2891
6319
4168
566
2926
14430
15715
23943
Left
14431
15716
23944
Left
14432
15717
23945
Left
14433
15718
23946
Right
14434
15719
23947
Right
4338
2892
6320
4169
566
2927
14435
15719
23948
Left
14436
15720
23949
Right
14437
15721
23950
Right
4339
2893
6321
4170
566
2928
14438
15721
23951
Left
14439
15722
23952
Left
14440
15723
23953
Right
14441
15724
23954
Right
4340
2894
6322
20227
566
5890
78555
15721
98740
Left
78556
69385
98741
Right
1277
447
566
6366
22346
3657641
Left
6367
2678344
3657642
Left
6368
2678345
3657643
Left
6369
15724
3657644
Right
6370
2678374
3657645
Right
6371
2678347
3657646
Right
6372
2678349
3657647
Right
1261
802864
488
78006
445
566
156695
15724
3657518
Left
156696
2678336
3657519
Right
156697
22344
3657520
Left
156698
22345
3657521
Right
24647
8388
382
109355
109694
566
14
false
525
632
16
regular
106058
15703
3657582
Right
186291
1090
715
1.6
1.6
0
2
92
479
481
1032
M423 524 C423 474 473 424 523 424 C1161 424 1989 424 2627 424 C2677 424 2727 474 2727 524 C2727 904 2727 1397 2727 1777 C2727 1827 2677 1877 2627 1877 C1989 1877 1161 1877 523 1877 C473 1877 423 1827 423 1777 C423 1397 423 904 423 524
1
true
6
2304.0
1453.0
369546
M126 226 C126 176 176 126 226 126 C1044 126 2109 126 2927 126 C2977 126 3027 176 3027 226 C3027 787 3027 1517 3027 2078 C3027 2128 2977 2178 2927 2178 C2109 2178 1044 2178 226 2178 C176 2178 126 2128 126 2078 C126 1517 126 787 126 226
1
true
6
2901.0
2052.0