Predicted to enable biotin binding activity; biotin-protein ligase activity; and enzyme binding activity. Predicted to be involved in histone biotinylation and response to biotin. 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). [provided by Alliance of Genome Resources, Apr 2022]