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Literature summary for 6.4.1.3 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)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Protein Variants

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

Localization

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

Metals/Ions

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

Molecular Weight [Da]

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

Purification (Commentary)

Purification (Comment) Organism
recombinant enzymes from Escherichia coli 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