792
Pathway
Taurine Metabolism I
Taurine is incorporated into the cytoplasm through a taurine ABC transporter. Once inside the cytoplasm, taurine interacts with an oxoglutaric acid and an oxygen through a taurine dioxygenase resulting in the release of succinic acid, sulfite , aminoacetaldehyde and carbon dioxide.
Metabolic
PW001028
Center
PathwayVisualizationContext1315
1513
2200
#000099
PathwayVisualization776
792
Taurine Metabolism I
Taurine is incorporated into the cytoplasm through a taurine ABC transporter. Once inside the cytoplasm, taurine interacts with an oxoglutaric acid and an oxygen through a taurine dioxygenase resulting in the release of succinic acid, sulfite , aminoacetaldehyde and carbon dioxide.
Metabolic
3
109909
686
SubPathway
109440
162
Compound
109910
544
SubPathway
109441
174
Compound
2499
9287300
Eichhorn E, van der Ploeg JR, Kertesz MA, Leisinger T: Characterization of alpha-ketoglutarate-dependent taurine dioxygenase from Escherichia coli. J Biol Chem. 1997 Sep 12;272(37):23031-6.
792
Pathway
2500
8808933
van der Ploeg JR, Weiss MA, Saller E, Nashimoto H, Saito N, Kertesz MA, Leisinger T: Identification of sulfate starvation-regulated genes in Escherichia coli: a gene cluster involved in the utilization of taurine as a sulfur source. J Bacteriol. 1996 Sep;178(18):5438-46.
792
Pathway
1
Cell
CL:0000000
6
Myocyte
CL:0000187
5
Hepatocyte
CL:0000182
2
Platelet
CL:0000233
3
Neuron
CL:0000540
4
Cardiomyocyte
CL:0000746
8
Beta cell
CL:0000639
7
Epithelial Cell
CL:0000066
1
Homo sapiens
9606
Eukaryote
Human
3
Escherichia coli
562
Prokaryote
18
Saccharomyces cerevisiae
4932
Eukaryote
Yeast
23
Pseudomonas aeruginosa
287
Prokaryote
12
Mus musculus
10090
Eukaryote
Mouse
5
Bos taurus
9913
Eukaryote
Cattle
17
Rattus norvegicus
10116
Eukaryote
Rat
24
Solanum lycopersicum
4081
Eukaryote
Tomato
21
Xenopus laevis
8355
Eukaryote
African clawed frog
4
Arabidopsis thaliana
3702
Eukaryote
Thale cress
49
Bathymodiolus platifrons
220390
Eukaryote
Deep sea mussel
60
Nitzschia sp.
0001
Eukaryote
Nitzschia4
10
Drosophila melanogaster
7227
Eukaryote
Fruit fly
6
Caenorhabditis elegans
6239
Eukaryote
Roundworm
2
Bacteria
2
Prokaryote
Bacteria
19
Schizosaccharomyces pombe
4896
Eukaryote
25
Escherichia coli (strain K12)
83333
Prokaryote
29
Saccharomyces cerevisiae (strain ATCC 204508 / S288c)
559292
Eukaryote
Baker's yeast
5
Cytoplasm
GO:0005737
31
Periplasmic Space
GO:0005620
1
Cytosol
GO:0005829
2
Mitochondrion
GO:0005739
3
Mitochondrial Matrix
GO:0005759
13
Endoplasmic Reticulum
GO:0005783
24
Mitochondrial Intermembrane Space
GO:0005758
4
Peroxisome
GO:0005777
10
Cell Membrane
GO:0005886
35
Chloroplast
GO:0009507
6
Lysosome
GO:0005764
16
Lysosomal Lumen
GO:0043202
7
Endoplasmic Reticulum Membrane
GO:0005789
11
Extracellular Space
GO:0005615
18
Melanosome Membrane
GO:0033162
14
Mitochondrial Outer Membrane
GO:0005741
36
Membrane
GO:0016020
12
Mitochondrial Inner Membrane
GO:0005743
25
Golgi apparatus
GO:0005794
15
Nucleus
GO:0005634
27
Peroxisome Membrane
GO:0005778
32
Inner Membrane
GO:0070258
20
Endoplasmic Reticulum Lumen
GO:0005788
21
Synapse
GO:0045202
53
Endoplasmic Reticulum Body
GO:0010168
34
Plant-Type Vacuole
GO:0000325
40
Periplasm
GO:0042597
19
sarcoplasmic reticulum
GO:0016529
1
Liver
BTO:0000759
72
9
9
Muscle
BTO:0000887
141
18
24
Brain
BTO:0000142
89
16
2
Endothelium
BTO:0000393
4
Adrenal Medulla
BTO:0000049
71
8
28
Stomach
BTO:0001307
155
26
7
Nervous System
BTO:0001484
18
Pancreas
BTO:0000988
25
Intestine
BTO:0000648
8
Blood Vessel
BTO:0001102
74
11
11
Heart
BTO:0000562
73
10
8
5
1
1
PW_BS000008
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1
1
1
PW_BS000029
31
1
5
1
1
PW_BS000031
107
31
3
PW_BS000107
108
1
3
PW_BS000108
188
1
18
PW_BS000024
187
31
18
PW_BS000024
318
31
23
PW_BS000024
315
1
23
PW_BS000024
336
1
12
1
PW_BS000028
129
1
5
12
1
PW_BS000129
111
5
12
1
PW_BS000111
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5
5
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1
5
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PW_BS000026
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1
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1
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PW_BS000054
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3
3
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PW_BS000103
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1
3
1
PW_BS000117
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1
17
1
PW_BS000118
132
1
12
1
PW_BS000132
147
1
24
1
PW_BS000147
155
3
24
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PW_BS000155
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2
24
1
PW_BS000157
161
3
18
1
PW_BS000161
178
3
21
1
PW_BS000178
85
24
10
1
1
PW_BS000085
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3
4
1
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2
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1
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35
4
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1
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5
18
1
PW_BS000024
289
2
49
1
PW_BS000024
253
5
4
1
PW_BS000024
133
3
12
1
PW_BS000133
112
2
12
1
PW_BS000112
345
24
12
1
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9
5
12
6
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PW_BS000115
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3
17
1
PW_BS000120
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2
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24
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PW_BS000016
14
10
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PW_BS000014
28
1
16
1
1
PW_BS000028
10
1
7
1
1
PW_BS000010
20
4
1
1
1
PW_BS000020
15
11
1
PW_BS000015
33
18
1
1
PW_BS000033
49
7
1
1
PW_BS000049
24
4
10
1
1
PW_BS000024
22
14
1
1
PW_BS000022
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1
2
1
PW_BS000013
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1
7
5
1
PW_BS000123
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13
5
1
PW_BS000125
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6
5
1
PW_BS000126
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1
16
5
1
PW_BS000127
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24
PW_BS000159
163
2
18
1
PW_BS000163
160
1
18
1
PW_BS000160
213
7
18
1
PW_BS000024
210
13
18
1
PW_BS000024
212
1
7
18
1
PW_BS000024
216
4
18
1
PW_BS000024
215
6
18
1
PW_BS000024
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10
18
PW_BS000024
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4
PW_BS000164
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36
4
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4
4
1
PW_BS000024
249
13
4
1
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291
6
49
1
PW_BS000024
292
4
49
1
PW_BS000024
298
1
7
10
1
PW_BS000024
300
13
10
1
PW_BS000024
301
6
10
1
PW_BS000024
302
1
16
10
1
PW_BS000024
223
12
4
1
PW_BS000024
17
12
1
1
PW_BS000017
293
4
1
PW_BS000024
294
11
4
1
PW_BS000024
134
12
12
1
PW_BS000134
130
13
12
1
PW_BS000130
331
7
12
1
PW_BS000028
332
1
7
12
1
PW_BS000028
113
6
12
1
PW_BS000113
115
10
12
PW_BS000115
337
1
16
12
1
PW_BS000028
341
4
1
12
1
PW_BS000028
114
11
12
PW_BS000114
329
14
12
1
PW_BS000028
343
18
12
1
PW_BS000028
360
4
10
12
1
PW_BS000028
405
10
5
PW_BS000115
409
11
5
PW_BS000115
415
18
5
1
PW_BS000115
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7
5
1
PW_BS000100
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4
10
5
1
PW_BS000115
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14
5
1
PW_BS000100
384
12
5
1
PW_BS000100
443
6
17
1
PW_BS000115
376
10
17
PW_BS000053
447
1
7
17
1
PW_BS000115
136
13
17
1
PW_BS000136
448
1
16
17
1
PW_BS000115
137
11
17
PW_BS000137
451
18
17
1
PW_BS000115
398
7
17
1
PW_BS000113
469
4
10
17
1
PW_BS000115
399
14
17
1
PW_BS000113
121
12
17
1
PW_BS000121
478
10
10
PW_BS000115
483
11
10
PW_BS000115
484
14
10
1
PW_BS000115
495
7
10
1
PW_BS000115
480
12
10
1
PW_BS000115
207
6
6
1
PW_BS000024
209
10
6
PW_BS000024
208
11
6
PW_BS000024
504
18
6
1
PW_BS000115
515
4
10
6
1
PW_BS000115
389
14
6
1
PW_BS000112
395
13
6
1
PW_BS000113
390
7
6
1
PW_BS000112
391
12
6
1
PW_BS000112
166
1
1
PW_BS000166
219
31
4
PW_BS000024
322
1
23
1
PW_BS000024
308
10
1
1
PW_BS000024
1
1
PW_BS000001
43
25
1
1
PW_BS000043
70
28
5
1
1
PW_BS000070
101
5
3
1
PW_BS000101
100
5
2
1
PW_BS000100
190
11
18
PW_BS000024
277
1
2
18
PW_BS000024
170
18
PW_BS000170
290
5
49
1
PW_BS000024
333
1
2
12
PW_BS000028
356
25
12
1
PW_BS000028
412
1
2
5
PW_BS000115
419
25
5
1
PW_BS000115
446
1
2
17
PW_BS000115
455
25
17
1
PW_BS000115
490
25
10
1
PW_BS000115
507
25
6
1
PW_BS000115
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
27
15
1
PW_BS000027
46
1
1
4
PW_BS000046
66
18
5
1
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72
5
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61
25
1
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51
8
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23
15
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PW_BS000023
91
8
5
1
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2
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7
1
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1
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2
1
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104
14
3
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143
1
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19
1
PW_BS000143
146
5
19
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199
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15
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218
15
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281
1
25
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10
4
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350
1
14
12
1
PW_BS000028
128
15
12
1
PW_BS000128
351
15
12
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353
25
12
7
PW_BS000028
335
27
12
1
PW_BS000028
184
1
2
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PW_BS000024
109
32
3
PW_BS000109
433
1
14
5
1
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410
15
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422
27
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15
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25
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485
15
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491
27
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499
15
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516
15
6
1
PW_BS000115
508
27
6
1
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517
15
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60
25
1
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36
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37
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21
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25
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10
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1
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180
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21
1
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152
8
4
PW_BS000152
214
25
18
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PW_BS000024
162
12
18
1
PW_BS000162
287
53
4
1
PW_BS000024
227
34
4
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65
11
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352
25
12
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370
2
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228
36
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232
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25
5
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25
17
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18
10
1
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1
7
6
1
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47
19
1
4
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313
23
PW_BS000024
241
5
29
PW_BS000024
257
15
29
1
PW_BS000024
304
1
2
PW_BS000024
195
13
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312
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171
Taurine
HMDB0000251
Taurine is a sulfur amino acid like methionine, cystine, cysteine and homocysteine. It is a lesser-known amino acid because it is not incorporated into the structural building blocks of protein. Yet taurine is an essential amino acid in pre-term and newborn infants of humans and many other species. Adults can synthesize their own taurine, yet are probably dependent in part on dietary taurine. Taurine is abundant in the brain, heart, breast, gallbladder and kidney and has important roles in health and disease in these organs. Taurine has many diverse biological functions serving as a neurotransmitter in the brain, a stabilizer of cell membranes and a facilitator in the transport of ions such as sodium, potassium, calcium and magnesium. Taurine is highly concentrated in animal and fish protein, which are good sources of dietary taurine. It can be synthesized by the body from cysteine when vitamin B6 is present. Deficiency of taurine occurs in premature infants and neonates fed formula milk, and in various disease states. Inborn errors of taurine metabolism have been described. OMIM 168605, an unusual neuropsychiatric disorder inherited in an autosomal dominant fashion through 3 generations of a family. Symptoms began late in the fifth decade in 6 affected persons and death occurred after 4 to 6 years. The earliest and most prominent symptom was mental depression not responsive to antidepressant drugs or electroconvulsive therapy. Sleep disturbances, exhaustion and marked weight loss were features. Parkinsonism developed later, and respiratory failure occurred terminally. OMIM 145350 describes congestive cardiomyopathy and markedly elevated urinary taurine levels (about 5 times normal). Other family members had late or holosystolic mitral valve prolapse and elevated urinary taurine values (about 2.5 times normal). In 2 with mitral valve prolapse, congestive cardiomyopathy eventually developed while the amounts of urinary taurine doubled. Taurine, after GABA, is the second most important inhibitory neurotransmitter in the brain. Its inhibitory effect is one source of taurine's anticonvulsant and antianxiety properties. It also lowers glutamic acid in the brain, and preliminary clinical trials suggest taurine may be useful in some forms of epilepsy. Taurine in the brain is usually associated with zinc or manganese. The amino acids alanine and glutamic acid, as well as pantothenic acid, inhibit taurine metabolism while vitamins A and B6, zinc and manganese help build taurine. Cysteine and B6 are the nutrients most directly involved in taurine synthesis. Taurine levels have been found to decrease significantly in many depressed patients. One reason that the findings are not entirely clear is because taurine is often elevated in the blood of epileptics who need it. It is often difficult to distinguish compensatory changes in human biochemistry from true metabolic or deficiency disease. Low levels of taurine are found in retinitis pigmentosa. Taurine deficiency in experimental animals produces degeneration of light-sensitive cells. Therapeutic applications of taurine to eye disease are likely to be forthcoming. Taurine has many important metabolic roles. Supplements can stimulate prolactin and insulin release. The parathyroid gland makes a peptide hormone called glutataurine (glutamic acid-taurine), which further demonstrates taurine's role in endocrinology. Taurine increases bilirubin and cholesterol excretion in bile, critical to normal gallbladder function. It seems to inhibit the effect of morphine and potentiates the effects of opiate antagonists. Low plasma taurine levels have been found in a variety of conditions, i.e., depression, hypertension, hypothyroidism, gout, institutionalized patients, infertility, obesity, kidney failure and others. (http://www.dcnutrition.com/AminoAcids/).
107-35-7
C00245
1123
15891
1091
DB01956
NCCS(O)(=O)=O
C2H7NO3S
InChI=1S/C2H7NO3S/c3-1-2-7(4,5)6/h1-3H2,(H,4,5,6)
XOAAWQZATWQOTB-UHFFFAOYSA-N
2-aminoethane-1-sulfonic acid
125.147
125.014663785
-0.08
2
taurine
0
0
FDB003191
1-aminoethane-2-sulfonate;1-aminoethane-2-sulfonic acid;2-aminoethanesulfonate;2-aminoethanesulfonic acid;2-aminoethylsulfonate;2-aminoethylsulfonic acid;2-sulfoethylamine;Aminoethylsulfonate;Aminoethylsulfonic acid;Taurine;B-aminoethylsulfonate;B-aminoethylsulfonic acid;Beta-aminoethylsulfonate;Beta-aminoethylsulfonic acid;Aminoethylsulphonate;Aminoethylsulphonic acid;2-aminoethanesulphonate;2-aminoethanesulphonic acid
PW_C000171
Taurine
1238
8
2826
29
2860
31
6349
107
6350
108
7354
188
7356
187
42374
318
42375
315
77642
336
77670
129
79015
111
120930
122
121684
429
121712
414
123496
135
124233
464
124263
450
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
1065
Oxygen
HMDB0001377
Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).
7782-44-7
C00007
977
15379
CPD-6641
952
O=O
O2
InChI=1S/O2/c1-2
MYMOFIZGZYHOMD-UHFFFAOYSA-N
oxidanone
31.9988
31.989829244
0
singlet oxygen
0
0
FDB022589
Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948
PW_C001065
O2
95
9
110
5
245
16
500
18
505
8
549
14
625
28
638
3
649
10
674
31
688
20
754
15
763
4
769
33
836
2
1375
49
2016
24
2531
22
2803
29
4260
42
4747
13
5467
123
5480
125
5493
126
5508
127
5809
108
5973
147
6129
159
7006
188
7032
163
7050
160
7319
213
7533
210
7560
212
8395
151
11816
216
11864
198
11883
215
11894
211
12057
225
12063
164
12247
286
12279
226
12325
249
12706
291
12716
292
13004
298
13016
300
13026
301
13038
302
13260
223
42276
17
42657
315
76910
293
77044
294
77214
134
77350
111
77363
130
77377
331
77395
332
77497
113
77512
115
77537
334
77626
336
77723
337
77736
112
77747
129
77756
341
77805
114
77812
133
78070
329
78151
132
78381
345
78805
343
79111
360
120047
408
120383
122
120426
405
120542
407
120553
414
120594
409
120601
406
120883
415
121045
124
121104
383
121605
434
121656
429
122117
382
122573
418
122689
384
122798
374
122822
443
123027
135
123060
376
123128
447
123139
136
123163
448
123176
119
123187
450
123219
137
123226
120
123459
451
123609
118
123669
398
124163
469
124214
464
124669
399
125145
454
125275
121
125425
482
125706
478
125731
483
125737
297
125740
479
125884
481
126100
299
126272
484
126522
495
126721
489
126825
480
126964
502
126986
207
127198
209
127214
208
127219
205
127222
501
127305
504
127345
206
127557
388
127574
515
127835
389
128081
395
128095
390
128312
506
128432
391
162
Sulfite
HMDB0000240
Endogenous sulfite is generated as a consequence of the body's normal processing of sulfur-containing amino acids. Sulfites occur as a consequence of fermentation and also occur naturally in a number of foods and beverages. As food additives, sulfiting agents were first used in 1664 and have been approved in the United States since the 1800s. Sulfite is an allergen, a neurotoxin, and a metabotoxin. An allergen is a compound that causes allergic reactions such as wheezing, rash, or rhinitis. A neurotoxin is a substance that causes damage to nerves or brain tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. As an allergen, sulfite is known to induce asthmatic reactions. Sulfite sensitivity occurs most often in asthmatic adults (predominantly women), but it is also occasionally reported in preschool children. Adverse reactions to sulfites in nonasthmatics are extremely rare. Asthmatics who are steroid-dependent or who have a higher degree of airway hyperreactivity may be at greater risk of experiencing a reaction to sulfite-containing foods. Sulfite sensitivity reactions vary widely, ranging from no reaction to severe. The majority of reactions are mild. These manifestations may include dermatologic, respiratory, or gastrointestinal signs and symptoms. The precise mechanisms of the sensitivity responses have not been completely elucidated: inhalation of sulfur dioxide (SO2) generated in the stomach following ingestion of sulfite-containing foods or beverages, a deficiency in a mitochondrial enzyme, and an IgE-mediated immune response have all been implicated. Exogenously supplied sulfite is detoxified by the enzyme sulfite oxidase. Sulfite oxidase (EC 1.8.3.1) is 1 of 3 enzymes in humans that require molybdenum as a cofactor. Under certain circumstances, chronically high levels of sulfite can lead to serious neurotoxicity. Sulfite oxidase deficiency (also called molybdenum cofactor deficiency) is a rare autosomal inherited disease that is typified by high concentrations of sulfite in the blood and urine. It is characterized by severe neurological symptoms such as untreatable seizures, attenuated growth of the brain, and mental retardation. It results from defects in the enzyme sulfite oxidase, which is responsible for the oxidation of sulfite to sulfate. This sulfite to sulfate reaction is the final step in the degradation of sulfur-containing metabolites (including the amino acids cysteine and methionine). The term "isolated sulfite oxidase deficiency" is used to define the deficiency caused by mutations in the sulfite oxidase gene. This differentiates it from another version of sulfite oxidase deficiency that is due to defects in the molybdenum cofactor biosynthetic pathway (with mutations in the MOCS1 or MOCS2 genes). Isolated sulfite oxidase deficiency is a rare but devastating neurologic disease that usually presents in early infancy with seizures and alterations in muscle tone (PMID: 16234925, 16140720, 8586770). Sulfite oxidase deficiency (as caused by MOCS1 or MOCS2) may be treated with cPMP, a precursor of the molybdenum cofactor (PMID: 20385644). The mechanism behind sulfite neurotoxicity appears to be related to its ability to bind and inhibit glutamate dehydrogenase (GDH). Inhibition of GDH leads to a decrease in alpha-ketoglutarate and a diminished flux through the tricarboxylic acid cycle. This is accompanied by a decrease in NADH through the mitochondrial electron transport chain, which leads to a decrease in mitochondrial membrane potential and in ATP synthesis. Since glutamate is a major metabolite in the brain, inhibition of GDH by sulfite appears to contribute to neural damage characteristic of sulfite oxidase deficiency in human infants (PMID: 15273247).
14265-45-3
C00094
1099
17359
SO3
1068
OS(O)=O
H2O3S
InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)
LSNNMFCWUKXFEE-UHFFFAOYSA-N
sulfurous acid
82.079
81.97246462
2
sulfurous acid
0
-1
FDB021915
Bisulfite;Sulfite dianion;Sulfite ion;Sulfite ions;Sulfonate;Sulfur trioxide;Sulfuric anhydride;Sulphite;Trioxosulfate(2-);Trioxosulfate(iv);Trioxosulphate(2-);Trioxosulphate(iv);[so3](2-);So3(2-);Sulphite ion
PW_C000162
Sulfite
713
8
1241
3
77784
111
79000
112
120563
122
120952
407
123197
135
123518
119
125893
481
127354
206
174
Succinic acid
HMDB0000254
Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021).
110-15-6
C00042
1110
15741
SUC
1078
DB00139
OC(=O)CCC(O)=O
C4H6O4
InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
KDYFGRWQOYBRFD-UHFFFAOYSA-N
butanedioic acid
118.088
118.02660868
0.25
2
succinic acid
0
-2
FDB001931
1,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;Ethylenesuccinate
PW_C000174
Succini
152
3
239
4
502
18
507
8
676
31
1265
5
4255
17
5383
103
6042
155
6102
161
6454
107
6455
108
6489
178
6764
117
6836
166
7362
163
7455
219
7456
220
7477
222
11866
198
12142
151
13259
223
42368
318
42369
315
42402
322
77143
133
77213
134
77483
111
77738
112
77749
129
78426
334
80024
368
80721
119
112846
308
113428
1
119984
406
120192
407
120385
122
120555
414
120990
408
122565
384
122767
120
123029
135
123189
450
123555
374
125138
121
125364
479
125549
481
125930
482
126713
480
126906
501
127082
206
127389
502
128304
391
40848
Aminoacetaldehyde
2-Aminoacetaldehyde belongs to the class of organic compounds known as monoalkylamines. These are organic compounds containing an primary aliphatic amine group. 2-Aminoacetaldehyde is soluble (in water) and an extremely weak acidic (essentially neutral) compound (based on its pKa).
17628
NCC=O
C2H5NO
InChI=1S/C2H5NO/c3-1-2-4/h2H,1,3H2
LYIIBVSRGJSHAV-UHFFFAOYSA-N
2-aminoacetaldehyde
59.0672
59.037113787
0.96
1
aminoacetaldehyde
0
1
Aminoacetaldehyde;;2-aminoacetaldehyde
PW_C040848
AmnActH
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
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
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
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
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
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
6574
Alpha-ketoglutarate-dependent taurine dioxygenase
P37610
Involved in oxidoreductase activity. Catalyzes the conversion of taurine and alpha ketoglutarate to sulfite, aminoacetaldehyde and succinate. Required for the utilization of taurine (2-aminoethanesulfonic acid) as an alternative sulfur source. Pentane-sulfonic acid, 3- (N-morpholino)propanesulfonic acid and 1,3-dioxo-2- isoindolineethanesulfonic acid are also substrates for this enzyme.
tauD
b0368
AP009048
3
1.14.11.17
7477
Taurine-binding periplasmic protein
Q47537
Involved in transporter activity. Part of a binding-protein-dependent transport system for taurine.
tauA
b0365
AP009048
3
6525
109
8626
232
7176
Taurine transport system permease protein tauC
Q47539
Involved in transporter activity. Part of a binding-protein-dependent transport system for taurine. Probably responsible for the translocation of the substrate across the membrane.
tauC
b0367
AP009048
3
6526
109
7429
Taurine import ATP-binding protein TauB
Q47538
Involved in nucleotide binding. Part of the ABC transporter complex TauABC involved in taurine import. Responsible for energy coupling to the transport system.
tauB
b0366
AP009048
3
3.6.3.36
6527
109
1262
taurine dioxygenase
3
PW_P001262
1456
6574
3694
taurine ABC transporter
3
PW_P003694
10583
7477
10584
7176
2
10585
7429
2
2558
false
PW_R002558
Right
9940
171
1
Compound
false
9941
134
1
Compound
false
9942
1065
1
Compound
false
9943
162
1
Compound
false
9944
174
1
Compound
false
9945
40848
1
Compound
false
9946
1316
1
Compound
false
2524
1262
1.14.11.17
166
PW_RCT000166
Right
981
166
171
Compound
1
107
982
166
414
Compound
1
108
983
166
1420
Compound
1
108
984
166
171
Compound
1
108
985
166
1034
Compound
1
108
986
166
1104
Compound
1
108
987
166
40034
Compound
1
108
166
26503
171
3
false
650
678
10
regular
100
100
26504
134
3
false
780
778
10
regular
100
110
26505
1065
65
false
810
604
10
regular
78
78
26506
162
3
false
1230
903
10
regular
100
100
26507
174
3
false
1230
478
10
regular
100
100
26508
40848
3
false
1305
673
10
regular
100
100
26509
1316
52
false
1150
599
10
regular
78
78
26511
414
108
42
false
499
936
10
regular
50
30
26512
1420
108
49
false
504
827
10
regular
78
78
26514
1034
108
43
false
498
646
10
regular
50
30
26515
1104
108
46
false
513
584
10
regular
44
43
26516
40034
108
55
false
492
497
10
regular
78
78
2678943
171
108
3
false
198
679
10
regular
100
100
12820
6574
2
false
944
693
8
subunit
regular
150
70
948268
7477
109
76
false
346
694
18
subunit
regular
150
70
948269
7176
109
77
false
341
664
8
subunit
regular
160
80
948270
7429
109
77
false
341
719
8
subunit
regular
160
80
9728
1262
776
12577
12820
803097
3694
776
109
944140
948268
944141
948269
944142
948270
39560
M750 728 C814 727 892 728 944 728
5
false
18
39561
M830 778 C830 733 877 728 944 728
5
false
18
39562
M849 682 C850 731 876 729 944 728
5
false
18
39563
M1280 903 C1280 820 1277 723 1094 728
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
39564
M1280 578 C1276 707 1254 724 1094 728
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
39565
M1305 723 C1252 722 1133 727 1094 728
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
39566
M1189 677 C1189 726 1163 728 1094 728
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3659534
M650 728 C615 729 524 729 496 729
5
false
18
3659535
M499 951 C448 951 420 801 421 764
5
false
7
3659536
M504 866 C479 866 419 838 421 764
5
false
7
3659537
M298 729 C343 728 289 729 346 729
5
false
18
false
false
3659538
M498 661 C468 661 420 651 421 694
5
false
7
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3659539
M513 605.5 C483 605.5 422 638 421 694
5
false
7
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3659540
M492 536 C462 536 422 629 421 694
5
false
7
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3661197
M1330 953 C1386 953 1389 953 1435 953
5
false
18
3661198
M1330 528 C1409 527 1402 528 1435 528
5
false
18
6907
776
2558
24916
26503
39560
Left
24917
26504
39561
Left
24918
26505
39562
Left
24919
26506
39563
Right
24920
26507
39564
Right
24921
26508
39565
Right
24922
26509
39566
Right
7151
2524
9728
1286
166
776
6407
26503
3659534
Left
6408
26511
3659535
Left
6409
26512
3659536
Left
6410
2678943
3659537
Right
6411
26514
3659538
Right
6412
26515
3659539
Right
6413
26516
3659540
Right
1269
803097
200
109581
109909
776
14
false
1435
918
16
regular
106273
26506
3661197
Left
109582
109910
776
14
false
1435
493
16
regular
106274
26507
3661198
Left
186382
1434
575
0.6
0.6
0
2
92
479
481
1487
M125 225 C125 175 175 125 225 125 C755 125 1445 125 1975 125 C2025 125 2075 175 2075 225 C2075 543 2075 958 2075 1276 C2075 1326 2025 1376 1975 1376 C1445 1376 755 1376 225 1376 C175 1376 125 1326 125 1276 C125 958 125 543 125 225
1
true
6
1950.0
1251.0
1488
M425 525 C425 475 475 425 525 425 C874 425 1327 425 1676 425 C1726 425 1776 475 1776 525 C1776 654 1776 821 1776 950 C1776 1000 1726 1050 1676 1050 C1327 1050 874 1050 525 1050 C475 1050 425 1000 425 950 C425 821 425 654 425 525
1
true
6
1351.0
625.0