Showing Metabocard for propanoyl-CoA (BASm0002653)

Common NamePropanoyl-coa
DescriptionPropionyl-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 ).
Structure
Molecular FormulaC24H40N7O17P3S
Average Mass823.59700
Monoisotopic Mass823.14142
IUPAC Name{[(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
Traditional Name[(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
CAS Registry Number317-66-8
SMILESCCC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(=O)([O-])OP(=O)([O-])OC[C@H]1O[C@@H](n2cnc3c(N)ncnc32)[C@H](O)[C@@H]1OP(=O)([O-])[O-]
InChI IdentifierInChI=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
InChI KeyQAQREVBBADEHPA-IEXPHMLFSA-N
CHEBI IDCHEBI:57392
HMDB IDHMDB0001275
Pathways
NameSMPDB/PathBank
Valine, leucine and isoleucine degradation
Propanoate metabolism
Primary bile acid biosynthesis
Oxidation of Branched Chain Fatty Acids
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
3-Methylglutaconic Aciduria Type I
3-Methylglutaconic Aciduria Type III
3-Methylglutaconic Aciduria Type IV
Beta-Ketothiolase Deficiency
Malonic Aciduria
Maple Syrup Urine Disease
Methylmalonic Aciduria
Methylmalonic Aciduria Due to Cobalamin-Related Disorders
Propionic Acidemia
3-Methylcrotonyl Coa Carboxylase Deficiency Type I
Isovaleric Aciduria
Congenital Bile Acid Synthesis Defect Type II
Cerebrotendinous Xanthomatosis (CTX)
Zellweger Syndrome
Familial Hypercholanemia (FHCA)
Congenital Bile Acid Synthesis Defect Type III
Methylmalonate Semialdehyde Dehydrogenase Deficiency
Phytanic Acid Peroxisomal Oxidation
Refsum Disease
Threonine and 2-Oxobutanoate Degradation
Malonyl-coa decarboxylase deficiency
3-hydroxyisobutyric acid dehydrogenase deficiency
3-hydroxyisobutyric aciduria
Isobutyryl-coa dehydrogenase deficiency
Isovaleric acidemia
Valproic Acid Metabolism Pathway
27-Hydroxylase Deficiency
StateSolid
Water Solubility4.27e+00 g/l
logP-0.31
logS-2.29
pKa (Strongest Acidic)0.83
pKa (Strongest Basic)4.95
Hydrogen Acceptor Count17
Hydrogen Donor Count9
Polar Surface Area363.63 Ų
Rotatable Bond Count21
Physiological Charge-4
Formal Charge0
Refractivity176.83 m³·mol⁻¹
Polarizability72.51

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