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Literature summary for 6.4.1.3 extracted from

  • Chloupkova, M.; Ravn, K.; Schwartz, M.; Kraus, J.P.
    Changes in the carboxyl terminus of the beta subunit of human propionyl-CoA carboxylase affect the oligomer assembly and catalysis: expression and characterization of seven patient-derived mutant forms of PCC in Escherichia coli (2000), Mol. Genet. Metab., 71, 623-632.
    View publication on PubMed
Search for more literature for 6.4.1.3

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
A513_R514insP insertion between residues 513 and 514 of the beta subunit, leads to partial degradation of the subunit, strongly reduced activity Homo sapiens
DELTA499 beta subunit, leads to partial degradation of the subunit, strongly reduced activity Homo sapiens
DELTA514 beta subunit, leads to partial degradation of the subunit, strongly reduced activity Homo sapiens
DELTA531 beta subunit, no effect Homo sapiens
L519P beta subunit, leads to complete degradation of the subunit, no activity Homo sapiens
N536D beta subunit, leads to complete degradation of the subunit, no activity Homo sapiens
R512C beta subunit, leads to partial degradation of the subunit, strongly reduced activity Homo sapiens

Localization

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

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
800000
-
 gel filtration 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
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
 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 + 6 * beta Homo sapiens

Cofactor

Cofactor Comment Organism Structure
biotin bound to enzyme Homo sapiens