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Description for Protein CPT1A

carnitine palmitoyltransferase 1A (liver)
2 total interacting proteins;
Icon Book Platelet Evidence (proteome studies/others : 4/0)
(membrane: 2; platelet: 3)
Summary:
The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq] (PubMed Links)
Domains and Motifs:
  • TM: Transmembrane domain
    • Gene Ontology:
    Gene Ontology Annotations
    KEGG - Enzyme ID(s):
    2.3.1.21
    KEGG - Orthology:
    K08765
    KEGG - Pathway(s):
    hsa00071; hsa03320; hsa04920
    (The yellow boxes represents platelet proteins)
    Nomenclature / Alternative Names:
    CPT 1, liver; CPT1; EC 2.3.1.21; CPT1-L; L-CPT1; Carnitine palmitoyltransferase 1A isoform 2; Carnitine palmitoyltransferase 1A isoform 1
    Approved Symbol:
    CPT1A
    (De-) Phosphorylations:
    Total (de-) phosphorylation sites: 2
    Human (de-) phosphorylation sites: 2; 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):

  • L-Carnitine(db);
  • Perhexiline(db)

    Associated Genetic Diseases:

  • Carnitine palmitoyltransferase IA deficiency(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 2 : 2
  • Isoform 1 : 2
    • Additional Identifiers:

    HPRD: 02755 Entrez Gene ID: 1374 OMIM ID: 600528 Swissprot Accession: P50416; Q8WZ48; B2RAQ8;