Propionyl-CoA carboxylase

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This article deals with molecular science, for other meanings of the acronym PCCA, see here.
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.[1]

Contents

Pathology

A deficiency is associated with propionic acidemia.

See also

References


External links

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 productionAcetyl-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 degradationAcyl 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
PhospholipidsDiacylglycerol kinase
SteroidsHMG-CoA reductase - Farnesyl-diphosphate farnesyltransferase - Squalene monooxygenase - Lanosterol synthase - CYP51A1
ProstanoidsCyclooxygenase - Thromboxane-A synthase - Prostacyclin synthase - Prostaglandin E synthase - Prostaglandin D2 synthase
LeukotrienesArachidonate 5-lipoxygenase - 5-Lipoxygenase activating protein - Leukotriene A4 hydrolase - Leukotriene C4 synthase
Bile acidsCholesterol 7 alpha-hydroxylase
v • d • e
Amino acid metabolism enzymes (essential in CAPS)
K
LYSINE→acetyl-CoA Saccharopine dehydrogenase - Glutaryl-CoA dehydrogenase
LEUCINE→acetyl-CoA Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase
TRYPTOPHAN→acetyl-CoA Kynureninase
PHENYLALANINE→tyrosine→acetoacetate (see below)
G
glycine→serine→pyruvate→TCA Serine hydroxymethyltransferase - Serine dehydratase
alanine→pyruvate→TCA Alanine transaminase
cysteine→pyruvate→TCA D-cysteine desulfhydrase
threonine→pyruvate→TCA L-threonine dehydrogenase
HISTIDINE→glutamate Histidine ammonia-lyase - Urocanate hydratase - Formiminotransferase cyclodeaminase
proline→glutamate Proline oxidase - Pyrroline-5-carboxylate reductase - 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 - PYCR1
arginine→glutamate Ornithine aminotransferase - Ornithine decarboxylase - Agmatinase
glutamate→alpha-ketoglutarate→TCA Glutamate dehydrogenase
METHIONINE→propionyl-CoA Methionine adenosyltransferase - Adenosylhomocysteinase - (or MTR/Homocysteine methyltransferase) - Cystathionine beta synthase - Cystathionine gamma-lyase
ISOLEUCINE/valine→propionyl-CoABranched-chain alpha-keto acid dehydrogenase complex
NT
HISTIDINE→histamine Histidine decarboxylase
tyrosine→dopamine→epinephrine Tyrosine hydroxylase - Aromatic L-amino acid decarboxylase - Dopamine beta hydroxylase - Phenylethanolamine N-methyltransferase - Catechol-O-methyl transferase
glutamate→GABA Glutamate decarboxylase
arginine→NO Nitric oxide synthase
Other
tyrosine→melanin Tyrosinase
TRYPTOPHAN→serotonin→melatonin Tryptophan hydroxylase - Aromatic L-amino acid decarboxylase - Acetylserotonin O-methyltransferase
cysteine+glutamate→glutathione Gamma-glutamylcysteine synthetase - Glutathione synthetase
glycine→creatine Guanidinoacetate N-methyltransferase - Creatine kinase
glutamate→glutamine Glutamine synthetase - Glutaminase
collagen Prolyl hydroxylase - Lysyl hydroxylase
see also disorders, intermediates

Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

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