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Description for Protein MCCC2

methylcrotonoyl-CoA carboxylase 2 (beta)
No Interacting Proteins
Icon Book Platelet Evidence (proteome studies/others : 1/0)
(platelet: 5)
Summary:
This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq] (PubMed Links)
Domains and Motifs:
  • SP: Signal Peptide
    • Gene Ontology:
    Gene Ontology Annotations
    KEGG - Enzyme ID(s):
    6.4.1.4
    KEGG - Orthology:
    K01969
    KEGG - Pathway(s):
    hsa00280; hsa01100
    (The yellow boxes represents platelet proteins)
    Nomenclature / Alternative Names:
    3-methylcrotonyl-CoA carboxylase 2; MCCB; 3-methylcrotonyl-CoA carboxylase, non biotin; Biotin carboxylase; Methylcrotonyl-CoA carboxylase, beta chain
    Approved Symbol:
    MCCC2
    (De-) Phosphorylations:
    Total (de-) phosphorylation sites: 1
    Human (de-) phosphorylation sites: 1; No platelet phosphorylation sites
    Phosphorylation Targets:
    Total phosphorylation targets: 0
    Human phosphorylation targets: 0;Predicted platelet targets: 0
    		Protein Characteristics:
    Isoform-specific Information
    Icon DrugsAssociated Drugs (DrugBank Accession):

  • Biotin(db)

    Associated Genetic Diseases:

  • 3-@methylcrotonyl-CoA carboxylase 2 deficiency(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 1 : 0
    • Additional Identifiers:

    HPRD: 01952 Entrez Gene ID: 64087 OMIM ID: 609014 Swissprot Accession: Q9HCC0;