Propionyl-CoA carboxylase

propionyl Coenzyme A carboxylase, alpha polypeptide
Identifiers
Symbol	PCCA
Entrez	5095
HUGO	8653
OMIM	232000
RefSeq	NM_000282
UniProt	P05165
Other data
EC number	6.4.1.3
Locus	Chr. 13 q32

propionyl Coenzyme A carboxylase, beta polypeptide
Identifiers
Symbol	PCCB
Entrez	5096
HUGO	8654
OMIM	232050
RefSeq	NM_000532
UniProt	P05166
Other data
EC number	6.4.1.3
Locus	Chr. 3 q21-q22

Propionyl-CoA carboxylase catalyses the carboxylation reaction of propionyl CoA. The product of the reaction is D-methylmalonyl CoA.

 ATP + propanoyl-CoA + HCO₃^- <=> ADP + phosphate + (S)-methylmalonyl-CoA

It has been classified both as a ligase^[1] and a lyase.^[2]

Pathology

A deficiency is associated with propionic acidemia.

v • d • e Ligases: carbon-carbon ligases (EC 6.4)
Pyruvate carboxylase - Acetyl-CoA carboxylase - Propionyl-CoA carboxylase - Methylcrotonyl-CoA carboxylase - Polyketide synthase

v • d • e Metabolism: lipid metabolism
Lipid production	Acetyl-CoA carboxylase - Fatty acid synthesis: Beta-ketoacyl-ACP synthase - Β-Ketoacyl ACP reductase - 3-Hydroxyacyl ACP dehydrase - Enoyl ACP reductase unsaturated (Enoyl CoA isomerase, 2,4 Dienoyl-CoA reductase) - odd chain (Propionyl-CoA carboxylase) - Fatty acid synthase - Long fatty acyl CoA synthetase
Lipid degradation	Acyl transport: Carnitine palmitoyltransferase I - Carnitine-acylcarnitine translocase - Carnitine palmitoyltransferase II Beta oxidation: Acyl CoA dehydrogenase (ACADL, ACADM, ACADS, ACADVL) - Enoyl-CoA hydratase - 3-Hydroxyacyl CoA dehydrogenase - Acetyl-CoA C-acyltransferase - Mitochondrial trifunctional protein Ketogenesis: HMG-CoA synthase - HMG-CoA lyase - Other: Malonyl-CoA decarboxylase
Phospholipids	Diacylglycerol kinase
Steroids	HMG-CoA reductase - Farnesyl-diphosphate farnesyltransferase - Squalene monooxygenase - Lanosterol synthase - CYP51A1
Prostanoids	Cyclooxygenase - Thromboxane-A synthase - Prostacyclin synthase - Prostaglandin E synthase - Prostaglandin D2 synthase
Leukotrienes	Arachidonate 5-lipoxygenase - 5-Lipoxygenase activating protein - Leukotriene A4 hydrolase - Leukotriene C4 synthase
Bile acids	Cholesterol 7 alpha-hydroxylase

v • d • e Amino acid metabolism enzymes
phenylalanine→tyrosine→fumarate→TCA	Phenylalanine hydroxylase - Tyrosine aminotransferase - 4-Hydroxyphenylpyruvate dioxygenase - Homogentisate 1,2-dioxygenase - Fumarylacetoacetate hydrolase
tyrosine→dopamine→epinephrine	Tyrosine hydroxylase - Aromatic L-amino acid decarboxylase - Dopamine beta hydroxylase - Phenylethanolamine N-methyltransferase
leucine→HMG-CoA (keto)	Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase
isoleucine/valine→propionyl-CoA	Branched-chain alpha-keto acid dehydrogenase complex
methionine→cysteine→propionyl-CoA	Methionine adenosyltransferase - Adenosylhomocysteinase - (or MTR) - Cystathionine beta synthase - Cystathionine gamma-lyase
propionyl-CoA→succinyl-CoA→TCA	Propionyl-CoA carboxylase - Methylmalonyl-CoA mutase
serine→glycine	Serine hydroxymethyltransferase
histidine	to histamine: Histidine decarboxylase - to glutamate: Histidine ammonia-lyase - Urocanate hydratase - Formiminotransferase cyclodeaminase
tryptophan→serotonin→melatonin	Tryptophan hydroxylase - Aromatic L-amino acid decarboxylase - 5-hydroxyindole-O-methyltransferase
asparagine→aspartate→oxaloacetate→TCA	Asparaginase/Asparagine synthetase - Aspartate transaminase
glutamate	Glutaminase - Glutamate decarboxylase
lysine→keto	Saccharopine dehydrogenase
other	Tyrosinase

This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Propionyl-CoA_carboxylase". A list of authors is available in Wikipedia.

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