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Literature summary 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

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

Localization

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

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
6.4.1.3 800000
-
 gel filtration 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
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
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 + 6 * beta Homo sapiens

Cofactor

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