BS8185

antibody from Bioworld Technology, Inc
Targeting: PPOX PPO, VP
Provider product page for BS8185
Western blot
Immunocytochemistry
Immunohistochemistry

Antibody data

Product number
BS8185 - Provider product page
Provider
Bioworld Technology, Inc
Product name
PPOX polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human PPOX.
Description
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.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
PPOX protein structure - BS8185 shown in red.