6.4.1.3: propionyl-CoA carboxylase
This is an abbreviated version!
For detailed information about propionyl-CoA carboxylase, go to the full flat file.
Word Map on EC 6.4.1.3
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6.4.1.3
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propionic
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acidemia
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biotin-dependent
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carboxylases
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methylmalonyl-coa
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acidosis
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methylmalonic
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3-hydroxypropionate
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methylcitrate
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propionylation
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hyperammonemia
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holocarboxylase
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3-methylcrotonyl-coa
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acidurias
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biotin-containing
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carboxyltransferase
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biotin-deficient
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hyperglycinemia
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beta-methylcrotonyl-coa
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biotin-binding
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odd-chain
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anaplerosis
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transcarboxylase
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3-hydroxyisovaleric
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propionylcarnitine
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synthesis
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analysis
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medicine
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apocarboxylases
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biotinidase
- 6.4.1.3
- propionic
- acidemia
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biotin-dependent
- carboxylases
- methylmalonyl-coa
- acidosis
-
methylmalonic
- 3-hydroxypropionate
-
methylcitrate
-
propionylation
-
hyperammonemia
- holocarboxylase
- 3-methylcrotonyl-coa
- acidurias
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biotin-containing
- carboxyltransferase
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biotin-deficient
- hyperglycinemia
- beta-methylcrotonyl-coa
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biotin-binding
-
odd-chain
-
anaplerosis
- transcarboxylase
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3-hydroxyisovaleric
- propionylcarnitine
- synthesis
- analysis
- medicine
- apocarboxylases
- biotinidase
Reaction
Synonyms
AccA3-PccB complex, acetyl-CoA/propionyl-CoA carboxylase, Carboxylase, propional coenzyme A (adenosine triphosphate-hydrolyzing), LA_2736-LA_2735, Pcase, PCC, PccA, PccA-1, PCCase, PccB, PccB-1, pccBC, PccE, Propanoyl-CoA:carbon dioxide ligase, Propionyl coenzyme A carboxylase, Propionyl coenzyme A carboxylase (adenosine triphosphate-hydrolyzing), Propionyl coenzyme A carboxylase (ATP-hydrolyzing), Propionyl-CoA carboxylase, propionyl-coenzyme A carboxylase
ECTree
6.4.1.3 propionyl-CoA carboxylaseAdvanced search results
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General Information on EC 6.4.1.3 - propionyl-CoA carboxylase
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GENERAL INFORMATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
malfunction
gene knockout/complementation demonstrates that the enzyme consists of a fusion protein of a biotin carboxylase and a biotin-carboxyl carrier protein (PccA, HFX_2490), a carboxyltransferase component (PccB, HFX_2478), and an essential small subunit (PccX, HFX_2479). Knockout of pccBX leads to an inability to utilize propionate and a higher intracellular propionyl-CoA level, indicating that the enzyme is indispensable for propionyl-CoA utilization. the pccBX-deleted strain displays multiple phenotypic changes, including retarded cell growth, decreased glucose consumption, impaired PHBV biosynthesis, and wrinkled cells. Genome-wide microarray analysis shows that many genes for glycolysis, pyruvate oxidation, PHBV accumulation, electron transport, and stress responses are affected in the pccBX-deleted strain
metabolism
physiological function
metabolism
methylmalonyl-CoA mutase and PCC are key enzmyes in the catabolic pathway of propionate metabolism in the developing and adult rat central nervous system
metabolism
PCC is essential for the catabolism of the amino acids L-Thr, L-Val, L-Ile and L-Met, cholesterol and fatty acids with an odd number of carbon atoms
metabolism
the enzyme is indispensability for propionyl-CoA assimilation und the global metabolism of Haloferax mediterranei
physiological function
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the PCC pathway is the dominant route for the supply of methylmalonyl-CoA for rapamycin production in Streptomyces hygroscopicus UV2-2 strain
physiological function
Q19842; Q20676
animal lifespan is significantly reduced relative to wild-type worms in mutant strains lacking either subunit PccA or PccB. Mitochondrial oxidative phosphorylation capacity and efficiency of isolated mitochondria are also significantly reduced in both mutant strains. In vivo quantitation of mitochondrial physiology is normal in PccB-1 mutants, but PccA-1 deletion mutants have significantly increased mitochondrial matrix oxidant burden as well as significantly decreased mitochondrial membrane potential and mitochondrial content. Both deletion strains have decreased accumulation of malate, isotopic enrichment in citrate, and increased lactate accumulation
physiological function
Streptomyces hygroscopicus ATCC 29253
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the PCC pathway is the dominant route for the supply of methylmalonyl-CoA for rapamycin production in Streptomyces hygroscopicus UV2-2 strain
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