Western Blot (WB)
(Western blot analysis of RPGR expression in Y79 (A), mouse heart (B) whole cell lysates.)
RPGR Polyclonal Antibody | anti-RPGR antibody
Anti-RPGR Antibody
Western Blot (WB)
(Western blot analysis of RPGR expression in Y79 (A), mouse heart (B) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of RPGR expression in Y79 (A), mouse heart (B) whole cell lysates.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. The encoded protein is found in the Golgi body and interacts with RPGRIP1. This protein localizes to the outer segment of rod photoreceptors and is essential for their viability. Mutations in this gene have been associated with X-linked retinitis pigmentosa (XLRP). Multiple alternatively spliced transcript variants that encode different isoforms of this gene have been reported, but the full-length natures of only some have been determined. [provided by RefSeq, Dec 2008]
Uniprot Description
RPGR: a guanine nucleotide exchange factor (GEF) that plays a role in cilia biogenesis and maintenance. Interacts with the GDP-bound form of the small GTPase RAB8A. Promotes the exchange of GDP to GTP, converting inactive GDP-bound Rab proteins into their active GTP-bound form. Probably regulates cilia formation by regulating actin stress filaments and cell contractility. Plays an important role in photoreceptor integrity. May play a critical role in spermatogenesis and in intraflagellar transport processes May be involved in microtubule organization and regulation of transport in primary cilia. Interacts with PDE6D, RPGRIP1, CEP290, and DFNB31. Defects in RPGR are the cause of retinitis pigmentosa (RP3, RP15, and RPDSI), cone-rod dystrophy, X-linked 1 (CORDX1), and macular degeneration, X-linked, atrophic (MDXLA). Six isoforms of the human protein are produced by alternative splicing. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments. Isoform 6 associates with microtubule organizing centers, cilium basal bodies, and cilium axonemes. Interacts with NPM1 (via C-terminus), RPGRIP1L, SMC1A and SMC3.
Protein type: GEFs, Rab; GEFs
Chromosomal Location of Human Ortholog: Xp21.1
Cellular Component: centrosome; Golgi apparatus; photoreceptor outer segment
Molecular Function: guanyl-nucleotide exchange factor activity; protein binding
Biological Process: cilium biogenesis; intracellular protein transport; intraflagellar transport; positive regulation of GTPase activity; response to stimulus; visual perception
Disease: Cone-rod Dystrophy, X-linked, 1; Macular Degeneration, X-linked Atrophic; Retinitis Pigmentosa 3; Retinitis Pigmentosa, X-linked, And Sinorespiratory Infections, With Or Without Deafness
Research Articles on RPGR
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Product Notes
The RPGR rpgr (Catalog #AAA8245501) is an Antibody and is intended for research purposes only. The product is available for immediate purchase. The Anti-RPGR Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's RPGR can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1/500 - 1/2000. Researchers should empirically determine the suitability of the RPGR rpgr for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "RPGR, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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