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Literature summary for 6.4.1.3 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)

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

Protein Variants

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

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, cholesterol 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, cholesterol and branched chain amino acids Homo sapiens ADP + phosphate + (S)-methylmalonyl-CoA
-
?

Subunits

Subunits Comment Organism
dodecamer 6 alpha and 6 beta subunits Homo sapiens

Cofactor

Cofactor Comment Organism Structure
biotin bound to enzyme Homo sapiens