Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
Product may be used with Western Blot (Transfected lysate).
Immunogen sequence: EASGCVLSQE LFQQRAQEAA ATQLGLKEMP SHCLVHLHKN CIPQYTLGHW QKLESARQFL TAHRLPLTLA GASYEGVAVN DCIESGRQAA VSVLGTEPNS
Protoporphyrinogen oxidase, the penultimate enzyme in the heme biosynthetic pathway, catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. The PPOX protein localizes to the inner membrane of mitochondria from various tissues, including heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Genetic deficiency of PPOX results in variegate porphyria, a low penetrance, autosomal dominant disorder characterized by cutaneous photosensitivity and/or various neurological manifestations. The rare homozygous variant of VP is characterized by severe PPOX deficiency, and results in the onset of photosensitization by porphyrins in early childhood, skeletal abnormalities of the hand, and, less constantly, short stature, mental retardation and convulsions.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: PPO; Protoporphyrinogen oxidase
Gene Aliases: PPO; PPOX; V290M; VP
UniProt ID: (Human) P50336
Entrez Gene ID: (Human) 5498
Molecular Function:
oxidase
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