Automated description from the Alliance of Genome Resources
Exhibits ATP-dependent peptidase activity; mitochondrial promoter sequence-specific DNA binding activity; and single-stranded DNA binding activity. Involved in proteolysis. Localizes to the mitochondrion. Human ortholog(s) of this gene implicated in CODAS syndrome. Is expressed in several structures, including adrenal medulla; alimentary system; genitourinary system; liver; and lung. Orthologous to human LONP1 (lon peptidase 1, mitochondrial).