Product nameAnti-PRPH2/RDS antibody
See all PRPH2/RDS primary antibodies
DescriptionRabbit polyclonal to PRPH2/RDS
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Recombinant fragment corresponding to Human PRPH2/RDS aa 124-248.
Database link: P23942
- WB: Over-expression Lysate (co-expressed with a C terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells) IHC-P: Human urinary bladder tissue
This product was previously labelled as PRPH2
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 59% PBS, 40% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab122057 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/250 - 1/500.|
|IHC-P||1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionMay 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.
Tissue specificityRetina (photoreceptor). In rim region of ROS (rod outer segment) disks.
Involvement in diseaseDefects in PRPH2 are the cause of retinitis pigmentosa type 7 (RP7) [MIM:608133]. 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 [MIM:136880].
Defects in PRPH2 are a cause of adult-onset vitelliform macular dystrophy (AVMD) [MIM:608161]. 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) [MIM:169150]. 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) [MIM:613105]. 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.
Note=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.
Sequence similaritiesBelongs to the PRPH2/ROM1 family.
- Information by UniProt
- AOFMD antibody
- AVMD antibody
- CACD2 antibody
ab122057, at 1/250, staining PRPH2/RDS in paraffin embedded Human retina tissue by Immunohistochemistry.
All lanes : Anti-PRPH2/RDS antibody (ab122057) at 1/250 dilution
Lane 1 : Negative control (vector only transfected HEK293T lysate)
Lane 2 : Over expression Lysate (co-expressed with a C terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells)
Developed using the ECL technique.
ab122057 has not yet been referenced specifically in any publications.