Automated description from the Alliance of Genome Resources
Predicted to have ATP binding activity. Involved in mitochondrial respiratory chain complex III assembly. Localizes to the mitochondrion. Human ortholog(s) of this gene implicated in Bjornstad syndrome; Leigh disease; and mitochondrial complex III deficiency nuclear type 1. Orthologous to human BCS1L (BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone).