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Literature summary 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

Cloned(Commentary)

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

Protein Variants

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

Localization

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

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.4.1.3 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

EC Number Organism UniProt Comment Textmining
6.4.1.3 Homo sapiens
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.4.1.3 ATP + propanoyl-CoA + HCO3-
-
 Homo sapiens ADP + phosphate + (S)-methylmalonyl-CoA
-
 ?
6.4.1.3 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

EC Number Subunits Comment Organism
6.4.1.3 dodecamer 6 * alpha + 6 * beta Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.4.1.3 biotin bound to enzyme Homo sapiens