holocarboxylase synthetase deficiency
Medical Definition
The neonatal form of multiple carboxylase deficiency that is caused by a defect or deficiency in holocarboxylase synthetase. hlcs is the enzyme that covalently links biotin to the biotin dependent carboxylases (propionyl-coa-carboxylase, pyruvate carboxylase, and beta-methylcrotonyl-coa carboxylase).
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