Thermo Fisher Scientific PPOX Monoclonal Antibody (2B5)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

-

ELISA (ELISA)

1:500-1:1,000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2a, kappa

Class

Monoclonal

Type

Antibody

Clone

2B5

Immunogen

PPOX (AAH02357, 378 a.a. approximately 477 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:PPOX,uniProtId:P50336-1,ncbiNodeId:9606,antigenRange:378-477,antigenLength:477,antigenImageFileName:H00005498-M04A_PPOX_P50336-1_House_mouse.svg,antigenImageFileNamePDP:H00005498-M04A_PPOX_P50336-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

See Label

Storage buffer

ascites

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: EASGCVLSQE LFQQRAQEAA ATQLGLKEMP SHCLVHLHKN CIPQYTLGHW QKLESARQFL TAHRLPLTLA GASYEGVAVN DCIESGRQAA VSVLGTEPNS

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.