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Invitrogen™ PPOX Polyclonal Antibody
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Catalog No. PIPA5117044
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PIPA5117044 100 μL
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Catalog No. PIPA5117044 Supplier Invitrogen™ Supplier No. PA5117044
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Description

Rabbit Polyclonal Antibody

Positive Samples: HepG2, Mouse spleen, Mouse liver, Mouse kidney Immunogen sequence: MGRTVVVLGG GISGLAASYH LSRAPCPPKV VLVESSERLG GWIRSVRGPN GAIFELGPRG IRPAGALGAR TLLLVSELGL DSEVLPVRGD HPAAQNRFLY VGGALHALPT GLRGLLRPSP PFSKPLFWAG LRELTKPRGK EPDETVHSFA QRRLGPEVAS LAMDSLCRGV FAGNSRELSI RSCFPSLFQA EQTHRSILLG LLLGAGRTPQ PDSALIRQAL AERWSQWSLR GGLEMLPQAL ETHLTSRGVS VLRGQPVCGL SLQAEGRWKV SLRDSSLEAD.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen PPOX
Applications ELISA, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.47 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene PPOX
Gene Accession No. P50336, P51175
Gene Alias PPO; Ppox; protoporhyrinogen oxidase; protoporphyrinogen oxidase; V290M; VP
Gene Symbols PPOX
Host Species Rabbit
Immunogen Recombinant protein (or fragment).
Purification Method Affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 19044, 5498
Target Species Human, Mouse
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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