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hlcs

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Official Full Name
holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Background
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.
Synonyms
HLCS; holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase); holocarboxylase synthetase (biotin (proprionyl Coenzyme A carboxylase (ATP hydrolysing)) ligase) , holocarboxylase synthetase (biotin [proprionyl Coenzyme A carboxylase (ATP hydrolysing)] ligase); biotin--protein ligase; HCS; Biotin [acetyl CoA carboxylase] ligase; Biotin [methylcrotonoyl CoA carboxylase] ligase; Biotin [methylmalonyl CoA carboxytransferase] ligase; Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase; Biotin apo protein ligase; Biotin apo-protein ligase; Biotin protein ligase; Biotin--[acetyl-CoA-carboxylase] ligase; BPL1_HUMAN; EC 6.3.4.-; Holocarboxylase synthetase; Holocarboxylase synthetase, EC 6.3.4; biotin--[methylcrotonoyl-CoA-carboxylase] ligase; biotin--[methylmalonyl-CoA-carboxytransferase] ligase; holocarboxylase synthetase (biotin-(proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)

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