Involved in brain development and regulation of mitochondrial ATP synthesis coupled proton transport. Located in mitochondrion. Is expressed in several structures, including brain; genitourinary system; gut gland; respiratory system; and skeletal muscle tissue. Human ortholog(s) of this gene implicated in Williams-Beuren syndrome and nuclear type mitochondrial complex I deficiency. Orthologous to human DNAJC30 (DnaJ heat shock protein family (Hsp40) member C30). [provided by Alliance of Genome Resources, Apr 2022]