<unk>  is a rare condition and form of isolated 17,20-lyase deficiency caused by deficiency in<unk> <unk> , a small hemoprotein that acts as an allosteric factor to facilitate the interaction of CYP17A1 (17α-hydroxylase/17,20-lyase) with P450 oxidoreductase (POR), thereby allowing for the 17,20-lyase activity of CYP17A1. The condition affects both adrenal and gonadal androgen biosynthesis and results in male pseudohermaphroditism. The principal biological role of<unk> <unk>  is reduction of methemoglobin, so<unk>  can also result in elevated methemoglobin levels and/or methemoglobinemia, similarly to deficiency of<unk> <unk>  reductase (methemoglobin reductase).
Cytochrome b5 deficiency
Multiple carboxylase deficiency
Succinic semialdehyde dehydrogenase deficiency
Coenzyme Q10 deficiency
6-phosphogluconate dehydrogenase deficiency
Glutaminase deficiency
Mitochondrial complex II deficiency
Tetrahydrobiopterin deficiency