Thermo Fisher Scientific PPOX Polyclonal Antibody, MaxPab

Applications

Tested Dilution

Publications

Western Blot (WB)

1-5 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

PPOX (NP_000300.1, 1 a.a. approximately 477 a.a) full-length human protein. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:PPOX,uniProtId:P50336-1,ncbiNodeId:9606,antigenRange:1-477,antigenLength:477,antigenImageFileName:H00005498-D01P_PPOX_P50336-1_Rabbit.svg,antigenImageFileNamePDP:H00005498-D01P_PPOX_P50336-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

See Label

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.4

Contains

no preservative

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Sequence of this protein is as follows: 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 HVISAIPASV LSELLPAEAA PLARALSAIT AVSVAVVNLQ YQGAHLPVQG FGHLVPSSED PGVLGIVYDS VAFPEQDGSP PGLRVTVMLG GSWLQTLEAS GCVLSQELFQ QRAQEAAATQ LGLKEMPSHC LVHLHKNCIP QYTLGHWQKL ESARQFLTAH RLPLTLAGAS YEGVAVNDCI ESGRQAAVSV LGTEPNS

Target Information

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.