RPGR, clone 7H12.1 | EMD Millipore MABN294 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

EMD Millipore

other brands :

Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava

product type :

antibody

product name :

RPGR, clone 7H12.1

catalog :

MABN294

quantity :

100 μg

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

7H12.1

reactivity :

human

application :

western blot, immunohistochemistry, immunohistochemistry - paraffin section

product information

Catalog Number :

MABN294

Subcategory :

Neuroscience

Product Name :

Anti-RPGR, clone 7H12.1

Product Type :

Antibodies

Clonality :

Monoclonal Antibody

Gene ID :

Q92834

Host Name :

Mouse

Antigen :

RPGR

Clone :

7H12.1

Conjugate :

Purified

Purification Method :

Protein G purified

Isotype :

IgG1κ

Product Description :

Anti-RPGR, clone 7H12.1

Cross Reactivity :

Human

Background :

The protein named X-linked retinitis pigmentosa GTPase regulator, or RP3/RPGR and encoded by the human gene named RPGR or RP3/XLRP3 is a ubiquitously expressed protein involved in vesicular transport associated processes that when defective gives rise to the X-linked retinitis pigmentosa type 3 (RP3), a severe, progressive and degenerative retinal dystrophy eventually leading to complete blindness. RPGR localizes in the retina to outer segment rod photoreceptors. RPGR is also a nucleotide exchange factor protein for small GTP-binding proteins such as Ran. RPGR directly interacts with the delta subunit of rod cyclic GMP phosphodiesterase and the enzyme is thought to regulate membrane insertion or solubilization of prenylated proteins including phosphodiesterase involved in phototransduction and small GTP-binding proteins of the Rab family. This suggests that RPGR mutations give rise to retinal degeneration by dysregulation of intracellular processes that determine protein localization and protein transport. Finally RPGR also plays a role in ciliogenesis, spermatogenesis and microtubule organization and regulation in primary cilia. There are multiple isoforms, but isoform 3 is found only in the retina; other tissues express other isoforms but all are believed to function similarly.

ALT Names :

X-linked retinitis pigmentosa GTPase regulator

Immunogen :

GST-tagged recombinant protein corresponding to human X-linked retinitis pigmentosa GTPase regulator.

Package Size :

100 μg

Uses :

Western Blotting, Immunohistochemistry (Paraffin)

Storage :

Stable for 1 year at 2-8°C from date of receipt