Automated description from the Alliance of Genome Resources (Release 7.4.0)
Enables biotin--[biotin carboxyl-carrier protein] ligase activity. Involved in biotin metabolic process. Located in mitochondrion. Is expressed in several structures, including genitourinary system; gut; nervous system; sensory organ; and thymus. Human ortholog(s) of this gene implicated in holocarboxylase synthetase deficiency. Orthologous to human HLCS (holocarboxylase synthetase).
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