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Holocarboxylase Synthetase Deficiency (Multiple Carboxylase Deficiency) Holocarboxylase synthetase is the enzyme that attaches biotin to the biotin-dependent carboxylases, acetyl-CoA carboxylase (ACC), propionyl-CoA carboxylase (PCC), pyruvate carboxylase (PC), and 3-methylcrotonyl-CoA carboxylase (3MCC)

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