Automated description from the Alliance of Genome Resources (Release 7.4.0)
Enables ATP hydrolysis activity. Acts upstream of or within mitochondrial respiratory chain complex III assembly. Located in mitochondrion. Is expressed in several structures, including alimentary system; extraembryonic component; genitourinary system; nervous system; and sensory organ. Used to study GRACILE syndrome and mitochondrial complex III deficiency nuclear type 1. Human ortholog(s) of this gene implicated in Bjornstad syndrome; GRACILE syndrome; and mitochondrial complex III deficiency nuclear type 1. Orthologous to human BCS1L (BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone).
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