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Literature summary extracted from

  • Chloupkova, M.; Maclean, K.N.; Alkhateeb, A.; Kraus, J.P.
    Propionic acidemia: analysis of mutant propionyl-CoA carboxylase enzymes expressed in Escherichia coli (2002), Hum. Mutat., 19, 629-640.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
6.4.1.3 expressed in Escherichia coli Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
6.4.1.3 A497V significantly increased activity Homo sapiens
6.4.1.3 A513_R514insP insertion, severe degradation of the subunit Homo sapiens
6.4.1.3 D178H increased alpha/beta subunit ratio, very low activity Homo sapiens
6.4.1.3 DELTA408 catalytically inactive Homo sapiens
6.4.1.3 DELTA499 severe degradation of the subunit Homo sapiens
6.4.1.3 DELTA514 severe degradation of the subunit Homo sapiens
6.4.1.3 E168K increased alpha/beta subunit ratio, very low activity Homo sapiens
6.4.1.3 G131R catalytically inactive Homo sapiens
6.4.1.3 G198D catalytically inactive Homo sapiens
6.4.1.3 L519P complete degradation of the subunit Homo sapiens
6.4.1.3 M442T catalytically inactive Homo sapiens
6.4.1.3 N536D complete degradation of the subunit Homo sapiens
6.4.1.3 P228L increased alpha/beta subunit ratio, very low activity Homo sapiens
6.4.1.3 R165W increased alpha/beta subunit ratio, very low activity Homo sapiens
6.4.1.3 R410W very low activity Homo sapiens
6.4.1.3 R44P catalytically inactive Homo sapiens
6.4.1.3 R512C severe degradation of the subunit Homo sapiens
6.4.1.3 S106R catalytically inactive Homo sapiens
6.4.1.3 V205D catalytically inactive Homo sapiens
6.4.1.3 V205D increased alpha/beta subunit ratio Homo sapiens

Localization

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

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
6.4.1.3 Mg2+ required to form a complex with ATP Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
6.4.1.3 800000
-
-
 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
-
 cDNA encoding for the alpha subunit
-
6.4.1.3 Homo sapiens P05166 cDNA encoding for the beta subunit
-

Purification (Commentary)

EC Number Purification (Comment) Organism
6.4.1.3 recombinant enzymes from Escherichia coli 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