Aliases for HLCS Gene
External Ids for HLCS Gene
Previous GeneCards Identifiers for HLCS Gene
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. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011]
GeneCards Summary for HLCS Gene
HLCS (Holocarboxylase Synthetase) is a Protein Coding gene. Diseases associated with HLCS include Holocarboxylase Synthetase Deficiency and Multiple Carboxylase Deficiency. Among its related pathways are Metabolism of water-soluble vitamins and cofactors and Metabolism. Gene Ontology (GO) annotations related to this gene include protein homodimerization activity and biotin binding.
UniProtKB/Swiss-Prot Summary for HLCS Gene
Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase.