Ppox - protoporphyrinogen oxidase

Enables oxygen-dependent protoporphyrinogen oxidase activity. Acts upstream of or within heme biosynthetic process. Is intrinsic component of mitochondrial inner membrane. Is expressed in several structures, including alimentary system; brain; genitourinary system; integumental system; and sensory organ. Used to study variegate porphyria. Human ortholog(s) of this gene implicated in variegate porphyria. Orthologous to human PPOX (protoporphyrinogen oxidase). [provided by Alliance of Genome Resources, Apr 2022]