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Literature summary extracted from

  • Muro, S.; Perez, B.; Desviat, L.R.; Rodriguez-Pombo, P.; Perez-Cerda, C.; Clavero, S.; Ugarte, M.
    Effect of PCCB gene mutations on the heteromeric and homomeric assembly of propionyl-CoA carboxylase (2001), Mol. Genet. Metab., 74, 476-483.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
6.4.1.3 expressed in COS-7 cells at 27°C and 37°C Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
6.4.1.3 A497V beta subunit, no effect on subunit interactions and activity Homo sapiens
6.4.1.3 DELTA531 beta subunit, strongly affects subunit interactions, very low activity Homo sapiens
6.4.1.3 E168K beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C), reduced activity at 37°C Homo sapiens
6.4.1.3 G131R beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C), reduced activity at 37°C Homo sapiens
6.4.1.3 G198D beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C) reduced activity at 37°C Homo sapiens
6.4.1.3 L519P beta subunit, strongly affects subunit interactions, very low activity Homo sapiens
6.4.1.3 N536D beta subunit, strongly affects subunit interactions, very low activity Homo sapiens
6.4.1.3 R165Q beta subunit, no effect on subunit interactions and activity Homo sapiens
6.4.1.3 R165W beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C), reduced activity at 37°C Homo sapiens
6.4.1.3 R512C beta subunit, strongly affects subunit interactions, very low activity Homo sapiens
6.4.1.3 R67S beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C), reduced activity at 37°C Homo sapiens
6.4.1.3 S106R beta subunit, no effect on subunit interactions only when expressed at low temperature (27°C), reduced activity at 37°C Homo sapiens

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, cholesterol 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, cholesterol 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 and 6 beta subunits Homo sapiens

Cofactor

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