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Description for Protein HLCS

holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
No Interacting Proteins
Icon Book Platelet Evidence (proteome studies/others : 0/1)
(platelet: 1)
Summary:
This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. (PubMed Links)
Domains and Motifs:
None Available
Gene Ontology:
Gene Ontology Annotations
KEGG - Enzyme ID(s):
6.3.4.10; 6.3.4.11; 6.3.4.15; 6.3.4.9
KEGG - Orthology:
K01942
KEGG - Pathway(s):
hsa00780; hsa01100
(The yellow boxes represents platelet proteins)
Nomenclature / Alternative Names:
HCS; Biotin apo protein ligase; Biotin-transcarboxylase synthetase; Biotin-acetyl-CoA-carboxylase ligase; Propionyl-CoA holocarboxylase synthetase; Biotin-[methylcrotonoyl-CoA-carboxylase] ligase; Acetyl-CoA carboxylase biotin holoenzyme synthetase; Holocarboxylase synthetase (biotin-[proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing) ligase
Approved Symbol:
HLCS
(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):

  • Biotin(db)

    Associated Genetic Diseases:

  • Holocarboxylase synthetase deficiency(Pd)
    		• Predicted Transmembrane Domains:
  • Isoform 1 : 0
    • Additional Identifiers:

    HPRD: 08360 Entrez Gene ID: 3141 OMIM ID: 609018 Swissprot Accession: P50747;