Western Blot (WB)
(mouse eye tissue were subjected to SDS PAGE followed by western blot with MBS7603055 (PRPH2 antibody) at dilution of 1:1000)
Rabbit PRPH2 Polyclonal Antibody | anti-PRPH2 antibody
PRPH2 Rabbit Polyclonal
Purity: > = 95% as determined by SDS-PAGE
Purity: > = 95% as determined by SDS-PAGE
IF: 1:20-1:200
Western Blot (WB)
(mouse eye tissue were subjected to SDS PAGE followed by western blot with MBS7603055 (PRPH2 antibody) at dilution of 1:1000)
Western Blot (WB)
(mouse eye tissue were subjected to SDS PAGE followed by western blot with MBS7603055 (PRPH2 antibody) at dilution of 1:1000)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is a member of the transmembrane 4 superfamily, also known as the tetraspanin family. Most of these members are cell-surface proteins that are characterized by the presence of four hydrophobic domains. The proteins mediate signal transduction events that play a role in the regulation of cell development, activation, growth and motility. This encoded protein is a cell surface glycoprotein found in the outer segment of both rod and cone photoreceptor cells. It may function as an adhesion molecule involved in stabilization and compaction of outer segment disks or in the maintenance of the curvature of the rim. This protein is essential for disk morphogenesis. Defects in this gene are associated with both central and peripheral retinal degenerations. Some of the various phenotypically different disorders are autosomal dominant retinitis pigmentosa, progressive macular degeneration, macular dystrophy and retinitis pigmentosa digenic. [provided by RefSeq, Jul 2008]
Uniprot Description
PRPH2: May function as an adhesion molecule involved in stabilization and compaction of outer segment disks or in the maintenance of the curvature of the rim. It is essential for disk morphogenesis. Defects in PRPH2 are the cause of retinitis pigmentosa type 7 (RP7). RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Defects in PRPH2 are a cause of retinitis punctata albescens (RPA). Defects in PRPH2 are a cause of adult-onset vitelliform macular dystrophy (AVMD). AVMD is a rare autosomal dominant disorder with incomplete penetrance and highly variable expression. Patients usually become symptomatic in the fourth or fifth decade of life with a protracted disease of decreased visual acuity. Defects in PRPH2 are a cause of patterned dystrophy of retinal pigment epithelium (PDREP). Patterned dystrophies of the retinal pigment epithelium (RPE) refer to a heterogeneous group of macular disorders. Three main types of PDREP have been described: reticular (fishnet-like) dystrophy, macroreticular (spider-shaped) dystrophy and butterfly-shaped pigment dystrophy. Defects in PRPH2 are a cause of choroidal dystrophy central areolar type 2 (CACD2). It is a disorder which affects the posterior pole of the eye, and early lesions consist of a non-specific area of granular hyperpigmentation at the fovea. The characteristic sign of the disorder, a zone of atrophy that develops in the macula of the eye and involves the retinal pigment epithelium and the choriocapillaris, occurs several decades after onset. Defects in PRPH2 are found in different retinal diseases including cone-rod dystrophy, retinitis pigmentosa, macular degeneration. The mutations underlying autosomal dominant retinitis pigmentosa and severe macular degeneration are largely missense or small in-frame deletions in a large intradiscal loop between the third and fourth transmembrane domains. In contrast, those associated with the milder pattern phenotypes or with digenic RP are scattered more evenly through the gene and are often nonsense mutations. This observation correlates with the hypothesis that the large loop is an important site of interaction between PRPH2 molecules and other protein components in the disk. Belongs to the PRPH2/ROM1 family.
Protein type: Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 6p21.1
Cellular Component: integral to membrane; integral to plasma membrane; photoreceptor outer segment
Biological Process: cell adhesion; cell surface receptor linked signal transduction; retina development in camera-type eye; visual perception
Disease: Choroidal Dystrophy, Central Areolar 2; Fundus Albipunctatus; Macular Dystrophy, Patterned, 1; Macular Dystrophy, Vitelliform, 3; Retinitis Pigmentosa 7
Research Articles on PRPH2
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Product Notes
The PRPH2 prph2 (Catalog #AAA7603055) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PRPH2 Rabbit Polyclonal reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's PRPH2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunofluorescence (IF). WB: 1:500-1:2000 IF: 1:20-1:200. Researchers should empirically determine the suitability of the PRPH2 prph2 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 "PRPH2, 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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