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Literature summary for 6.4.1.3 extracted from

  • Muro, S.; Perez, B.; Rodriguez-Pombo, P.; Desviat, L.R.; Perez-Cerda, C.; Ugarte, M.
    Mutations affecting the beta-beta homomeric interaction in propionic acidaemia: An approach to the determination of the beta-propionyl-CoA carboxylase functional domains (2000), J. Inherit. Metab. Dis., 23, 300-304.
    View publication on PubMed
Search for more literature for 6.4.1.3

Cloned(Commentary)

Cloned (Comment) Organism
expressed using a two hybrid system in COS cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
A487V no effect on activity Homo sapiens
DELTA531 very low activity Homo sapiens
L519P very low activity Homo sapiens
R512C very low activity Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + propanoyl-CoA + HCO3- Homo sapiens key enzyme in catabolic pathways for odd-chain fatty acids and branched chain amino acids ADP + phosphate + (S)-methylmalonyl-CoA
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + propanoyl-CoA + HCO3-
-
 Homo sapiens ADP + phosphate + (S)-methylmalonyl-CoA
-
 ?
ATP + propanoyl-CoA + HCO3- key enzyme in catabolic pathways for odd-chain fatty acids and branched chain amino acids Homo sapiens ADP + phosphate + (S)-methylmalonyl-CoA
-
 ?

Subunits

Subunits Comment Organism
dodecamer 6 * alpha + 6 * beta Homo sapiens

Cofactor

Cofactor Comment Organism Structure
biotin bound to enzyme Homo sapiens