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Rabbit Anti-RPGRIP1 Antibody (CSB-PA850326ESR2HU)
Supplier: CUSABIO BIOTECH CO.
X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 Antibody,RPGR-interacting protein 1 Antibody,RPGRIP1 AntibodyMore alternative names for the antibody
CORD13 antibody|LCA6 antibody|Retinitis pigmentosa GTPase regulator interacting protein 1 antibody|RGI1 antibody|RGRIP antibody|RPGR interacting protein antibody|RPGR interacting protein 1 antibody|RPGR1_HUMAN antibody|RPGRIP antibody|RPGRIP1d antibody|X linked retinitis pigmentosa GTPase regulator interacting protein 1 antibody
Anti-RPGRIP1 antibody (ab135832)
Anti-RPGRIP1 antibody (ab135832)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 protein (400-580AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein function|| |
May function as scaffolding protein. Required for normal location of RPGR at the connecting cilium of photoreceptor cells. Required for normal disk morphogenesis and disk organization in the outer segment of photoreceptor cells and for survival of photoreceptor cells. .
|Protein tissue specificity|| |
Strong expression in retina, with weaker expression in testis. Expressed in other neurons such as amacrine cells. Colocalizes with RGPR in the outer segment of rod photoreceptors and cone outer segments. .
|Involvement in disease|| |
Leber congenital amaurosis 6 (LCA6) [MIM:613826]: A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Cone-rod dystrophy 13 (CORD13) [MIM:608194]: An inherited retinal dystrophy characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa, due to cone photoreceptors degenerating at a higher rate than rod photoreceptors. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Note=Heterozygous non-synonymous variants of RPGRIP1 may cause or increase the susceptibility to various forms of glaucoma, a genetically heterogeneous disorder. It is the second cause of blindness worldwide owing to the progressive degeneration of retinal ganglion neurons (PubMed:21224891). .
|Protein sequence and domain|| |
Belongs to the RPGRIP1 family. The C2 domain does not bind calcium ions, and does not bind phosphoinositides.
|Protein cellular localization|| |
Cell projection, cilium . Note=Situated between the axonemal microtubules and the plasma membrane. .
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CUSABIO BIOTECH CO.
|Product type|| |
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