Automated description from the Alliance of Genome Resources (Release 7.0.0)
Enables several functions, including ATP hydrolysis activity; ATP-dependent peptidase activity; and DNA binding activity. Acts upstream of or within negative regulation of insulin receptor signaling pathway; proteolysis; and regulation of peptidyl-tyrosine phosphorylation. Located in mitochondrion. Is expressed in several structures, including adrenal medulla; genitourinary system; gut; lung; and submandibular gland primordium. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial).