1826
Pathway
Propanoyl-CoA Degradation
The degradation of propanoyl-CoA starts with propanoyl-CoA undergoing a decarboxylase reaction by reacting with hydrogen carbonate and ATP resulting in the release of a phosphate, an ADP, a hydrogen ion and an S-methylmalonyl-CoA. This compound in turn reacts through an epimerase reaction resulting in the release of a R-methylmalonyl-CoA. This compound in turn can undergo a reversible reaction through a methylmalonyl-CoA mutase resulting in the release of a succinyl-CoA. This compound can be converted back to R-methylmalonyl-CoA through a methylmalonyl-CoA mutase.
Methylmalonyl-CoA can then be converted into propanoyl-CoA through a methylmalonyl CoA decarboxylase . This compound in turn reacts with a succinate through a propionyl-CoA succinate CoA transferase resulting in the release of a propanoate and a succinyl-CoA.
Metabolic
PW002057
Center
PathwayVisualizationContext2344
1501
1700
#000099
PathwayVisualization1810
1826
Propanoyl-CoA Degradation
The degradation of propanoyl-CoA starts with propanoyl-CoA undergoing a decarboxylase reaction by reacting with hydrogen carbonate and ATP resulting in the release of a phosphate, an ADP, a hydrogen ion and an S-methylmalonyl-CoA. This compound in turn reacts through an epimerase reaction resulting in the release of a R-methylmalonyl-CoA. This compound in turn can undergo a reversible reaction through a methylmalonyl-CoA mutase resulting in the release of a succinyl-CoA. This compound can be converted back to R-methylmalonyl-CoA through a methylmalonyl-CoA mutase.
Methylmalonyl-CoA can then be converted into propanoyl-CoA through a methylmalonyl CoA decarboxylase . This compound in turn reacts with a succinate through a propionyl-CoA succinate CoA transferase resulting in the release of a propanoate and a succinyl-CoA.
Metabolic
3
3823
4554901
Kay WW: Genetic control of the metabolism of propionate by Escherichia coli K12. Biochim Biophys Acta. 1972 May 16;264(3):508-21.
1826
Pathway
3825
10769117
Haller T, Buckel T, Retey J, Gerlt JA: Discovering new enzymes and metabolic pathways: conversion of succinate to propionate by Escherichia coli. Biochemistry. 2000 Apr 25;39(16):4622-9.
1826
Pathway
1
Cell
CL:0000000
2
Platelet
CL:0000233
5
Hepatocyte
CL:0000182
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
17
Rattus norvegicus
10116
Eukaryote
Rat
12
Mus musculus
10090
Eukaryote
Mouse
24
Solanum lycopersicum
4081
Eukaryote
Tomato
18
Saccharomyces cerevisiae
4932
Eukaryote
Yeast
21
Xenopus laevis
8355
Eukaryote
African clawed frog
4
Arabidopsis thaliana
3702
Eukaryote
Thale cress
60
Nitzschia sp.
0001
Eukaryote
Nitzschia4
5
Bos taurus
9913
Eukaryote
Cattle
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
49
Bathymodiolus platifrons
220390
Eukaryote
Deep sea mussel
23
Pseudomonas aeruginosa
287
Prokaryote
29
Saccharomyces cerevisiae (strain ATCC 204508 / S288c)
559292
Eukaryote
Baker's yeast
3
Mitochondrial Matrix
GO:0005759
5
Cytoplasm
GO:0005737
2
Mitochondrion
GO:0005739
1
Cytosol
GO:0005829
11
Extracellular Space
GO:0005615
14
Mitochondrial Outer Membrane
GO:0005741
15
Nucleus
GO:0005634
4
Peroxisome
GO:0005777
13
Endoplasmic Reticulum
GO:0005783
7
Endoplasmic Reticulum Membrane
GO:0005789
10
Cell Membrane
GO:0005886
27
Peroxisome Membrane
GO:0005778
31
Periplasmic Space
GO:0005620
35
Chloroplast
GO:0009507
12
Mitochondrial Inner Membrane
GO:0005743
32
Inner Membrane
GO:0070258
19
sarcoplasmic reticulum
GO:0016529
34
Plant-Type Vacuole
GO:0000325
24
Mitochondrial Intermembrane Space
GO:0005758
36
Membrane
GO:0016020
53
Endoplasmic Reticulum Body
GO:0010168
25
Golgi apparatus
GO:0005794
28
Stomach
BTO:0001307
155
26
2
Endothelium
BTO:0000393
1
Liver
BTO:0000759
72
9
7
Nervous System
BTO:0001484
18
Pancreas
BTO:0000988
25
Intestine
BTO:0000648
8
Blood Vessel
BTO:0001102
74
11
4
Adrenal Medulla
BTO:0000049
71
8
4
3
1
1
PW_BS000004
8
5
1
1
PW_BS000008
3
2
1
1
PW_BS000003
2
1
1
1
PW_BS000002
15
11
1
PW_BS000015
70
28
5
1
1
PW_BS000070
103
3
3
1
PW_BS000103
120
3
17
1
PW_BS000120
133
3
12
1
PW_BS000133
155
3
24
1
PW_BS000155
161
3
18
1
PW_BS000161
178
3
21
1
PW_BS000178
222
3
4
1
PW_BS000024
224
2
4
1
PW_BS000024
112
2
12
1
PW_BS000112
132
1
12
1
PW_BS000132
111
5
12
1
PW_BS000111
368
3
60
1
PW_BS000028
406
3
5
1
PW_BS000115
407
2
5
1
PW_BS000115
122
5
5
1
PW_BS000122
124
1
5
1
PW_BS000124
119
2
17
1
PW_BS000119
135
5
17
1
PW_BS000135
118
1
17
1
PW_BS000118
479
3
10
1
PW_BS000115
297
5
10
1
PW_BS000024
299
1
10
1
PW_BS000024
481
2
10
1
PW_BS000115
501
3
6
1
PW_BS000115
205
5
6
1
PW_BS000024
388
1
6
1
PW_BS000112
206
2
6
1
PW_BS000024
16
2
1
2
PW_BS000016
22
14
1
1
PW_BS000022
13
1
2
1
PW_BS000013
32
1
15
1
5
PW_BS000032
5
4
1
1
PW_BS000005
39
7
1
1
3
PW_BS000039
18
13
1
1
PW_BS000018
10
1
7
1
1
PW_BS000010
49
7
1
1
PW_BS000049
14
10
1
PW_BS000014
58
1
14
1
1
PW_BS000058
59
27
1
1
PW_BS000059
27
15
1
PW_BS000027
46
1
1
4
PW_BS000046
29
1
1
1
PW_BS000029
66
18
5
1
8
PW_BS000066
72
5
1
3
PW_BS000072
61
25
1
7
PW_BS000061
51
8
1
PW_BS000051
23
15
1
1
PW_BS000023
31
1
5
1
1
PW_BS000031
91
8
5
1
1
PW_BS000091
54
1
3
1
5
PW_BS000054
89
2
PW_BS000089
26
1
1
1
5
PW_BS000026
7
1
1
PW_BS000007
97
1
5
2
1
PW_BS000097
100
5
2
1
PW_BS000100
104
14
3
1
PW_BS000104
101
5
3
1
PW_BS000101
117
1
3
1
PW_BS000117
129
1
5
12
1
PW_BS000129
108
1
3
PW_BS000108
143
1
5
19
1
PW_BS000143
146
5
19
1
PW_BS000146
107
31
3
PW_BS000107
147
1
24
1
PW_BS000147
151
1
4
1
PW_BS000151
166
1
1
PW_BS000166
188
1
18
PW_BS000024
160
1
18
1
PW_BS000160
199
14
18
1
PW_BS000024
210
13
18
1
PW_BS000024
213
7
18
1
PW_BS000024
211
10
18
PW_BS000024
198
5
18
1
PW_BS000024
216
4
18
1
PW_BS000024
217
15
18
PW_BS000024
218
15
18
1
PW_BS000024
163
2
18
1
PW_BS000163
190
11
18
PW_BS000024
225
35
4
1
PW_BS000024
277
1
2
18
PW_BS000024
170
18
PW_BS000170
281
1
25
1
PW_BS000024
164
4
PW_BS000164
285
10
4
1
PW_BS000024
226
4
4
1
PW_BS000024
290
5
49
1
PW_BS000024
223
12
4
1
PW_BS000024
308
10
1
1
PW_BS000024
315
1
23
PW_BS000024
322
1
23
1
PW_BS000024
318
31
23
PW_BS000024
253
5
4
1
PW_BS000024
134
12
12
1
PW_BS000134
329
14
12
1
PW_BS000028
333
1
2
12
PW_BS000028
336
1
12
1
PW_BS000028
332
1
7
12
1
PW_BS000028
350
1
14
12
1
PW_BS000028
128
15
12
1
PW_BS000128
351
15
12
PW_BS000028
353
25
12
7
PW_BS000028
335
27
12
1
PW_BS000028
115
10
12
PW_BS000115
130
13
12
1
PW_BS000130
331
7
12
1
PW_BS000028
334
4
12
1
PW_BS000028
184
1
2
1
PW_BS000024
1
1
PW_BS000001
94
3
PW_BS000094
109
32
3
PW_BS000109
382
14
5
1
PW_BS000100
412
1
2
5
PW_BS000115
429
1
5
1
PW_BS000115
123
1
7
5
1
PW_BS000123
433
1
14
5
1
PW_BS000115
408
4
5
1
PW_BS000115
410
15
5
1
PW_BS000115
125
13
5
1
PW_BS000125
383
7
5
1
PW_BS000100
405
10
5
PW_BS000115
422
27
5
1
PW_BS000115
435
15
5
PW_BS000115
399
14
17
1
PW_BS000113
446
1
2
17
PW_BS000115
464
1
17
1
PW_BS000115
447
1
7
17
1
PW_BS000115
468
1
14
17
1
PW_BS000115
374
4
17
1
PW_BS000053
444
15
17
1
PW_BS000115
136
13
17
1
PW_BS000136
398
7
17
1
PW_BS000113
376
10
17
PW_BS000053
472
25
17
7
PW_BS000115
375
27
17
1
PW_BS000053
470
15
17
PW_BS000115
484
14
10
1
PW_BS000115
485
15
10
1
PW_BS000115
300
13
10
1
PW_BS000024
495
7
10
1
PW_BS000115
478
10
10
PW_BS000115
491
27
10
1
PW_BS000115
499
15
10
PW_BS000115
389
14
6
1
PW_BS000112
516
15
6
1
PW_BS000115
395
13
6
1
PW_BS000113
390
7
6
1
PW_BS000112
209
10
6
PW_BS000024
508
27
6
1
PW_BS000115
517
15
6
PW_BS000115
47
19
1
4
PW_BS000047
6
1
3
1
PW_BS000006
212
1
7
18
1
PW_BS000024
227
34
4
1
PW_BS000024
241
5
29
PW_BS000024
257
15
29
1
PW_BS000024
17
12
1
1
PW_BS000017
304
1
2
PW_BS000024
293
4
1
PW_BS000024
384
12
5
1
PW_BS000100
391
12
6
1
PW_BS000112
121
12
17
1
PW_BS000121
298
1
7
10
1
PW_BS000024
513
1
7
6
1
PW_BS000115
313
23
PW_BS000024
42
24
1
1
PW_BS000042
105
11
3
PW_BS000105
157
2
24
1
PW_BS000157
159
24
PW_BS000159
152
8
4
PW_BS000152
187
31
18
PW_BS000024
219
31
4
PW_BS000024
220
1
4
PW_BS000024
162
12
18
1
PW_BS000162
195
13
18
PW_BS000024
249
13
4
1
PW_BS000024
286
36
4
1
PW_BS000024
287
53
4
1
PW_BS000024
294
11
4
1
PW_BS000024
312
5
23
1
PW_BS000024
320
11
23
PW_BS000024
114
11
12
PW_BS000114
327
1
1
12
5
PW_BS000028
347
1
3
12
5
PW_BS000028
345
24
12
1
PW_BS000028
310
31
2
PW_BS000024
409
11
5
PW_BS000115
424
1
1
5
5
PW_BS000115
425
1
3
5
5
PW_BS000115
418
24
5
1
PW_BS000115
137
11
17
PW_BS000137
459
1
1
17
5
PW_BS000115
460
1
3
17
5
PW_BS000115
454
24
17
1
PW_BS000115
483
11
10
PW_BS000115
489
24
10
1
PW_BS000115
480
12
10
1
PW_BS000115
208
11
6
PW_BS000024
506
24
6
1
PW_BS000115
482
4
10
1
PW_BS000115
502
4
6
1
PW_BS000115
20
4
1
1
1
PW_BS000020
43
25
1
1
PW_BS000043
341
4
1
12
1
PW_BS000028
356
25
12
1
PW_BS000028
414
1
5
5
1
PW_BS000115
419
25
5
1
PW_BS000115
450
1
5
17
1
PW_BS000115
455
25
17
1
PW_BS000115
490
25
10
1
PW_BS000115
507
25
6
1
PW_BS000115
463
Hydrogen carbonate
HMDB0000595
Bicarbonate, or hydrogen carbonate, is a simple single carbon molecule that plays surprisingly important roles in diverse biological processes. Among these are photosynthesis, the Krebs cycle, whole-body and cellular pH regulation, and volume regulation. Since bicarbonate is charged it is not permeable to lipid bilayers. Mammalian membranes thus contain bicarbonate transport proteins to facilitate the specific transmembrane movement of HCO3(-). Bicarbonate ion is an anion that consists of one central carbon atom surrounded by three oxygen atoms in a trigonal planar arrangement, with a hydrogen atom attached to one of the oxygens. The bicarbonate ion carries a negative one formal charge and is the conjugate base of carbonic acid, H2CO3. The carbonate radical is an elusive and strong one-electron oxidant. Bicarbonate in equilibrium with carbon dioxide constitutes the main physiological buffer. The bicarbonate-carbon dioxide pair stimulates the oxidation, peroxidation and nitration of several biological targets. The demonstration that the carbonate radical existed as an independent species in aqueous solutions at physiological pH and temperature renewed the interest in the pathophysiological roles of this radical and related species. The carbonate radical has been proposed to be a key mediator of the oxidative damage resulting from peroxynitrite production, xanthine oxidase turnover and superoxide dismutase1 peroxidase activity. The carbonate radical has also been proposed to be responsible for the stimulatory effects of the bicarbonate-carbon dioxide pair on oxidations mediated by hydrogen peroxide/transition metal ions. The ultimate precursor of the carbonate radical anion being bicarbonate, carbon dioxide, peroxymonocarbonate or complexes of transition metal ions with bicarbonate-derived species remains a matter of debate. The carbonate radical mediates some of the pathogenic effects of peroxynitrite. The carbonate radical as the oxidant produced from superoxide dismutase (EC 1.15.1.1, SOD1) peroxidase activity. Peroxymonocarbonate is a biological oxidant, whose existence is in equilibrium with hydrogen peroxide and bicarbonate. (PMID: 17505962, 17215880).
71-52-3
C00288
769
17544
HCO3
749
OC([O-])=O
CHO3
InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)/p-1
BVKZGUZCCUSVTD-UHFFFAOYSA-M
carbonic acid
61.0168
60.992568898
0.57
2
carbonic acid
0
-1
FDB022134
Bicarbonate;Bicarbonate (hco3-);Bicarbonate anion;Bicarbonate ion;Bicarbonate ion (hco31-);Bicarbonate ions;Carbonate;Carbonate (hco31-);Carbonate ion (hco31-);Carbonic acid;Hydrocarbonate(1-);Hydrogen carbonate;Hydrogen carbonate (hco3-);Hydrogen carbonate anion;Hydrogen carbonate ion;Hydrogen carbonate ion (hco3-);Hydrogencarbonate;Hydrogentrioxocarbonate;Monohydrogen carbonate;[co2(oh)](-);Acid carbonate;Hco3(-);Hydrogen carbonic acid;Acid carbonic acid;Bicarbonic acid;Bicarbonic acid ion
PW_C000463
HCO3
22
4
1687
8
2393
3
2397
2
2613
15
3145
70
5391
103
5445
120
5571
133
6049
155
6110
161
6494
178
7482
222
9092
224
77959
112
78630
132
78762
111
80029
368
119993
406
121209
407
121436
122
121557
124
123779
119
123994
135
124115
118
125372
479
126059
297
126360
299
126541
481
126914
501
127511
205
127922
388
128114
206
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
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
1560
S-Methylmalonyl-CoA
HMDB0002310
S-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. S-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). S-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, S-methylmalonyl-CoA is primarily located in the cytoplasm, mitochondria and peroxisome. In humans, S-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the propanoate metabolism pathway, and the valine, leucine and isoleucine degradation pathway. S-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the isovaleric aciduria pathway, the maple syrup urine disease pathway, isobutyryl-CoA dehydrogenase deficiency, and the 3-methylglutaconic aciduria type III pathway. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).
73173-91-8
C00683
21252287
D-METHYL-MALONYL-COA
13628334
C[C@@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
C25H40N7O19P3S
InChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16+,17+,18-,22?/m0/s1
MZFOKIKEPGUZEN-JDVCRUKVSA-N
(2S)-3-[(2-{3-[(2R)-3-[({[({[(3S,4R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid
867.607
867.131252359
-2.39
10
(2S)-3-[(2-{3-[(2R)-3-{[({[(3S,4R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid
0
-5
FDB022959
(s)-methylmalonyl-coa;(s)-methylmalonyl-coenzyme a
PW_C001560
S-MmCoA
2286
4
2695
3
78623
133
79032
112
121578
407
122251
406
124135
119
124804
120
126414
479
126730
481
127979
501
128322
206
1523
R-Methylmalonyl-CoA
HMDB0002255
R-Methylmalonyl-CoA belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain. R-Methylmalonyl-CoA is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). R-Methylmalonyl-CoA has been primarily detected in urine. Within the cell, R-methylmalonyl-CoA is primarily located in the cytoplasm. In humans, R-methylmalonyl-CoA is involved in the threonine and 2-oxobutanoate degradation pathway, the valine, leucine and isoleucine degradation pathway, and the propanoate metabolism pathway. R-Methylmalonyl-CoA is also involved in several metabolic disorders, some of which include the maple syrup urine disease pathway, the 3-methylglutaconic aciduria type IV pathway, 3-methylcrotonyl CoA carboxylase deficiency type I, and 3-hydroxyisobutyric acid dehydrogenase deficiency. Methylmalonyl-CoA is an intermediate in the metabolism of Propanoate. It is a substrate for Malonyl-CoA decarboxylase (mitochondrial), Methylmalonyl-CoA mutase (mitochondrial) and Methylmalonyl-CoA epimerase (mitochondrial).
73173-92-9
C01213
22833590
METHYL-MALONYL-COA
17216177
C[C@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
C25H40N7O19P3S
InChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12-,13?,16?,17?,18+,22?/m1/s1
MZFOKIKEPGUZEN-YLYUOEEYSA-N
(2R)-3-[(2-{3-[(2R)-3-[({[({[5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid
867.607
867.131252359
-2.39
10
(2R)-3-[(2-{3-[(2R)-3-{[({[5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido]propanamido}ethyl)sulfanyl]-2-methyl-3-oxopropanoic acid
0
-5
FDB022930
PW_C001523
R-MmCoA
2290
4
2697
2
4387
3
78624
133
79035
132
79204
112
121580
407
122252
406
122578
124
124138
119
124805
120
125150
118
126415
479
126733
299
127980
501
128326
388
808
Succinyl-CoA
HMDB0001022
Succinyl-CoA is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase (EC 1.2.4.2) through decarboxylation, and is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (EC 6.2.1.5). Succinyl-CoA may be an end product of peroxisomal beta-oxidation of dicarboxylic fatty acids; the identification of an apparently specific succinyl-CoA thioesterase (ACOT4, EC 3.1.2.3, hydrolyzes succinyl-CoA) in peroxisomes strongly suggests that succinyl-CoA is formed in peroxisomes. Acyl-CoA thioesterases (ACOTs) are a family of enzymes that catalyze the hydrolysis of the CoA esters of various lipids to the free acids and coenzyme A, thereby regulating levels of these compounds. (PMID: 16141203).
604-98-8
C00091
439161
15380
3-METHYLBENZYLSUCCINYL-COA
388307
CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=O
C25H40N7O19P3S
InChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20?,24-/m1/s1
VNOYUJKHFWYWIR-FZEDXVDRSA-N
4-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-4-oxobutanoic acid
867.607
867.131252359
-2.35
10
4-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-4-oxobutanoic acid
0
-5
FDB022375
Coa s-(hydrogen succinate);Coa s-succinate;Coenzyme a s-(hydrogen succinate);Coenzyme a s-succinate;S-(hydrogen butanedioate;S-(hydrogen butanedioate) coa;S-(hydrogen butanedioate) coenzyme a;S-(hydrogen butanedioic acid;S-succinoylcoenzyme a;Suc-co-a;Suc-coa;Succ-coa;Succ-coenzyme a;Succ-s-coa;Succ-s-coenzyme a;Succ-s-coenzyme-a;Succ-coenzyme-a;Succino-1-yl-coenzyme a;Succinyl coa;Succinyl coenzyme a;Succinyl-s-coa;Succinyl-s-coenzyme a;Succinyl-s-coenzyme-a;Succinylcoenzyme-a;Succinylcoenzyme a
PW_C000808
Suc-CoA
233
4
1055
3
3669
2
5378
103
6039
155
6097
161
6485
178
7015
160
7361
163
7474
222
77140
133
78101
112
78576
132
80021
368
119978
406
120769
407
122014
124
122763
120
123365
119
124568
118
125358
479
126164
299
126306
481
126901
501
127868
206
1401
Adenosylcobalamin
HMDB0002086
Adenosylcobalamin is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase. A cobalamin (cbl) derivative in which the substituent is deoxyadenosyl. It is one of two metabolically active forms synthesized upon ingestion of vitamin B12 and is the predominant form in the liver; it acts as a coenzyme in the reaction catalyzed by methylmalonyl-CoA mutase (MCM; E.C. 5.4.99.2). Inborn errors of vitamin B12 metabolism are autosomal recessive disorders and have been classified into nine distinct complementation classes. Disorders affecting adenosylcobalamin cause methylmalonic acidemia and metabolic acidosis. Methylmalonyl-CoA mutase catalyzes the conversion of L-methylmalonyl-CoA to succinyl-CoA and uses adenosylcobalamin (AdoCbl) as a cofactor. Cbl must be transported into mitochondria, reduced and adenosylated before it can be utilized by MCM. (PMID: 17011224).
13870-90-1
C00194
18408
ADENOSYLCOBALAMIN-5-P
30791458
C[C@@]12[C@H](C3N4C1=C(C1=[N]5C(=CC6=[N]7[Co+]45([N]4=CN(C5=C4C=C(C(C)=C5)C)[C@H]4O[C@H](CO)[C@@H](OP([O-])(O[C@@H](CNC(=O)CC2)C)=O)[C@H]4O)([N]2=C([C@H]([C@](C)(CC(=O)N)[C@]32C)CCC(=O)N)C(C)=C7[C@](C)([C@@H]6CCC(=O)N)CC(=O)N)C[C@@H]2[C@@H](O)[C@@H](O)[C@H](N3C4=C(C(N)=NC=N4)N=C3)O2)C([C@@H]1CCC(=O)N)(C)C)C)CC(N)=O
C72H100CoN18O17P
InChI=1S/C62H90N13O14P.C10H12N5O3.Co/c1-29-20-39-40(21-30(29)2)75(28-70-39)57-52(84)53(41(27-76)87-57)89-90(85,86)88-31(3)26-69-49(83)18-19-59(8)37(22-46(66)80)56-62(11)61(10,25-48(68)82)36(14-17-45(65)79)51(74-62)33(5)55-60(9,24-47(67)81)34(12-15-43(63)77)38(71-55)23-42-58(6,7)35(13-16-44(64)78)50(72-42)32(4)54(59)73-56;1-4-6(16)7(17)10(18-4)15-3-14-5-8(11)12-2-13-9(5)15;/h20-21,23,28,31,34-37,41,52-53,56-57,76,84H,12-19,22,24-27H2,1-11H3,(H15,63,64,65,66,67,68,69,71,72,73,74,77,78,79,80,81,82,83,85,86);2-4,6-7,10,16-17H,1H2,(H2,11,12,13);/q;;+2/p-2/t31-,34-,35-,36-,37+,41-,52-,53-,56?,57+,59-,60+,61+,62+;4-,6-,7-,10-;/m11./s1
ZIHHMGTYZOSFRC-QRVZQHAISA-L
(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate
1579.5818
1578.65834557
12
(10S,12R,13S,17R,23R,24R,25R,30S,35S,36S,40S,41S,42R,46R)-1-{[(2S,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl}-30,35,40-tris(2-carbamoylethyl)-24,36,41-tris(carbamoylmethyl)-46-hydroxy-12-(hydroxymethyl)-5,6,17,23,28,31,31,36,38,41,42-undecamethyl-15,20-dioxo-11,14,16-trioxa-2lambda5,9,19,26,43lambda5,44lambda5,45lambda5-heptaaza-15lambda5-phospha-1-cobaltadodecacyclo[27.14.1.1^{1,34}.1^{2,9}.1^{10,13}.0^{1,26}.0^{3,8}.0^{23,27}.0^{25,42}.0^{32,44}.0^{39,43}.0^{37,45}]heptatetraconta-2(47),3,5,7,27,29(44),32,34(45),37,39(43)-decaene-2,43,44,45-tetrakis(ylium)-1,1,1-triuid-15-olate
0
0
FDB022837
(5'-deoxy-5'-adenosyl)cobamide coenzyme;5'-deoxy-5'-adenosyl vitamin b12;5'-deoxy-5'-adenosylcobalamin;Adenosylcobalamin 5'-phosphate;Calomide;Cobalamin coenzyme;Cobamamide;Cobamamide 5'-phosphate;Cobamide coenzyme;Coenzyme b12;Deoxyadenosylcobalamin;Dibencozide;Funacomide;Vitamin b12 coenzyme;Vitamin b12 coenzymes;Adenosylcob(iii)alamin;5,6-dimethylbenzimidazolyl-5-deoxyadenosyl-cobamide;(5,6-dimethylbenzimidazolyl)cobamide coenzyme;Alpha-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;5'-deoxy-5'-adenosyl-5,6-dimethylbenzimidazolylcobamide;5,6-dimethylbenzimidazolyl-co-5'-deoxy-5'-adenosylcobamide;Dmbc coenzyme;A-(5,6-dimethylbenzimidazolyl)cobamide coenzyme;α-(5,6-dimethylbenzimidazolyl)cobamide coenzyme
PW_C001401
Adnscbn
2401
3
78628
112
121582
407
124140
119
126419
481
127985
206
988
Propionyl-CoA
HMDB0001275
Propionyl-CoA is an intermediate in the metabolism of propanoate. Propionic aciduria is caused by an autosomal recessive disorder of propionyl coenzyme A (CoA) carboxylase deficiency (EC 6.4.1.3). In propionic aciduria, propionyl CoA accumulates within the mitochondria in massive quantities; free carnitine is then esterified, creating propionyl carnitine, which is then excreted in the urine. Because the supply of carnitine in the diet and from synthesis is limited, such patients readily develop carnitine deficiency as a result of the increased loss of acylcarnitine derivatives. This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. Propionyl-CoA is a substrate for Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acetyl-coenzyme A synthetase 2-like (mitochondrial), Propionyl-CoA carboxylase alpha chain (mitochondrial), Methylmalonate-semialdehyde dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Malonyl-CoA decarboxylase (mitochondrial), Acetyl-coenzyme A synthetase (cytoplasmic), 3-ketoacyl-CoA thiolase (mitochondrial) and Propionyl-CoA carboxylase beta chain (mitochondrial). (PMID: 10650319).
317-66-8
C00100
439164
15539
PROPIONYL-COA
388310
CCC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
C24H40N7O17P3S
InChI=1S/C24H40N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,34-35H,4-10H2,1-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19?,23-/m1/s1
QAQREVBBADEHPA-UXYNFSPESA-N
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy({3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy})phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
823.597
823.141423115
-2.29
9
[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid
0
-4
FDB022529
2-methylacetyl-coa;2-methylacetyl-coenzyme a;Propanoyl-coa;Propanoyl-coenzyme a;Propionyl-coa;Propionyl-coenzyme a;Alpha-methylacetyl-coa;Alpha-methylacetyl-coenzyme a
PW_C000988
PropCoA
1277
8
1694
3
2285
4
2445
5
4914
13
9091
224
77641
334
78436
112
78556
111
78636
133
120995
122
121576
407
121681
408
122266
406
123560
135
124133
119
124231
374
124819
120
125935
297
126430
479
126557
482
126568
481
127395
205
127997
501
128130
502
128141
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
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
159
Propionic acid
HMDB0000237
Propionic acid (PA) is widely used as an antifungal agent in food. It is present naturally at low levels in dairy products and occurs ubiquitously, together with other short-chain fatty acids (SCFA), in the gastro-intestinal tract of humans and other mammals as an end-product of the microbial digestion of carbohydrates. It has significant physiological activity in animals. PA is irritant but produces no acute systemic effects and has no demonstrable genotoxic potential. (PMID 1628870) Propionic aciduria is one of the most frequent organic acidurias, a disease that comprise many various disorders. The outcome of patients born with Propionic aciduria is poor intellectual development patterns, with 60% having an IQ less than 75 and requiring special education. Successful liver and/or renal transplantations, in a few patients, have resulted in better quality of life but have not necessarily prevented neurological and various visceral complications. These results emphasize the need for permanent metabolic follow-up whatever the therapeutic strategy. (PMID 15868474) Decreased early mortality, less severe symptoms at diagnosis, and more favorable short-term neurodevelopmental outcome were recorded in patients identified through expanded newborn screening. (PMID 16763906).
79-09-4
C00163
1032
30768
PROPIONATE
1005
DB03766
CCC(O)=O
C3H6O2
InChI=1S/C3H6O2/c1-2-3(4)5/h2H2,1H3,(H,4,5)
XBDQKXXYIPTUBI-UHFFFAOYSA-N
propanoic acid
74.0785
74.036779436
0.68
1
propanoic acid
0
-1
FDB008285
Adofeed;Antischim b;Carboxyethane;Ethanecarboxylate;Ethanecarboxylic acid;Ethylformate;Ethylformic acid;Luprosil;Metacetonate;Metacetonic acid;Methylacetate;Methylacetic acid;Monoprop;Propanate;Propanoate;Propanoic acid;Propcorn;Propionate;Propkorn;Prozoin;Pseudoacetate;Pseudoacetic acid;Toxi-check;Acide propanoique;Acide propionique;Ch3-ch2-cooh;Pa;Propioic acid;Propionsaeure;Propoic acid;Propioate;Propoate
PW_C000159
3:0
2307
4
4563
18
6363
107
6364
108
42468
318
42469
315
78639
133
79228
130
121285
125
122272
406
123856
136
124825
120
126434
479
128003
501
6463
Protein sbm
P27253
Involved in isomerase activity. Specific function unknown.
sbm
b2917
AP009048
3
5.4.99.2
10202
Methylmalonyl-CoA decarboxylase
P52045
Catalyzes the decarboxylation of methylmalonyl-CoA to propionyl-CoA. Could be part of a pathway that converts succinate to propionate.
scpB
25
4.1.1.41
7313
Uncharacterized protein ygfH
P52043
Involved in catalytic activity. Specific function unknown.
ygfH
b2920
AP009048
3
2.8.3.-
135967
304
3725
methylmalonyl-CoA mutase
3
PW_P003725
10621
6463
2
536
1401
3726
methylmalonyl-CoA decarboxylase
3
PW_P003726
10622
10202
6
3724
propionyl-CoA:succinate CoA transferase
3
PW_P003724
10620
7313
6007
PW_R006007
Right
24547
1560
1
Compound
false
24548
1523
1
Compound
false
6006
false
PW_R006006
Both
24545
808
1
Compound
false
24546
1523
1
Compound
false
5968
3725
6008
false
PW_R006008
Right
24549
1523
1
Compound
false
24550
40034
1
Compound
false
24551
988
1
Compound
false
24552
1316
1
Compound
false
5969
3726
6009
false
PW_R006009
Both
24553
988
1
Compound
false
24554
174
1
Compound
false
24555
159
1
Compound
false
24556
808
1
Compound
false
5970
3724
6005
PW_R006005
Right
24538
988
1
Compound
false
24539
463
1
Compound
false
24540
414
1
Compound
false
24541
1104
1
Compound
false
24542
1034
1
Compound
false
24543
40034
1
Compound
false
24544
1560
1
Compound
false
70651
463
47
false
496
314
10
regular
78
78
70652
414
42
false
610
347
10
regular
50
30
70653
1104
46
false
793
470
10
regular
44
43
70654
1034
43
false
775
348
10
regular
50
30
70655
40034
55
false
876
303
10
regular
78
78
70656
1560
3
false
1000
361
10
regular
100
120
70660
1523
3
false
1250
360
10
regular
100
120
70661
808
3
false
1250
1105
10
regular
100
100
70662
1401
9
false
1300
598
10
regular
100
25
70666
1523
3
false
390
1095
10
regular
100
120
70667
1401
9
false
910
1122
10
regular
100
25
70675
40034
55
false
307
921
10
regular
78
78
70676
988
3
false
391
575
10
regular
100
100
70677
1316
52
false
306
691
10
regular
78
78
70678
174
3
false
526
490
10
regular
100
100
70679
159
3
false
1006
575
10
regular
100
100
32435
6463
6
false
1220
588
8
subunit
regular
160
80
32437
6463
6
false
870
1117
8
subunit
regular
160
80
32441
10202
17
false
350
795
8
subunit
regular
180
85
948356
7313
304
2
false
653
592
8
subunit
regular
150
70
27285
3725
1810
32116
32435
3757
70662
100434
Cofactor
27287
3725
1810
32118
32437
3758
70667
100441
Cofactor
27291
3726
1810
32122
32441
803154
3724
1810
304
944226
948356
100430
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