Thermo Fisher Scientific PPOX Monoclonal Antibody (2B5)

Applications

Western Blot (WB)

• Tested Dilution: 1:500–1:1,000

ELISA

• Tested Dilution: 1:500–1:1,000

Product Specifications

Product Specific Information

Sequence of this protein is as follows:
EASGCVLSQE LFQQRAQEAA ATQLGLKEMP SHCLVHLHKN CIPQYTLGHW QKLESARQFL TAHRLPLTLA GASYEGVAVN DCIESGRQAA VSVLGTEPNS

Target Information

Protoporphyrinogen oxidase (PPOX) is the penultimate enzyme in the heme biosynthetic pathway and catalyzes the 6-electron oxidation of protoporphyrinogen IX to protoporphyrin IX.
PPOX localizes to the inner mitochondrial membrane in various tissues including heart, brain, placenta, lung, liver, skeletal muscle, kidney, and pancreas.
Genetic deficiency of PPOX leads to variegate porphyria, an autosomal dominant disorder characterized by cutaneous photosensitivity and/or neurological symptoms.
Rare homozygous variants cause severe PPOX deficiency with early childhood onset of photosensitization, skeletal abnormalities, and sometimes short stature, mental retardation, and convulsions.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.