Automated description from the Alliance of Genome Resources (Release 3.2)
Exhibits RNA binding activity and single-stranded DNA binding activity. Involved in negative regulation of mitochondrial RNA catabolic process and regulation of mitochondrial translation. Localizes to mitochondrion; nucleus; and ribonucleoprotein complex. Is expressed in several structures, including brain; genitourinary system; heart; liver; and skeletal muscle. Human ortholog(s) of this gene implicated in French Canadian Leigh disease and Leigh disease. Orthologous to human LRPPRC (leucine rich pentatricopeptide repeat containing).