Anti-RPGRIP1 Antibody

$99.00$319.00

Reactivity: Human
Applications: ELISA,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-RPGRIP1 Antibody (CSB-PA850326ESR2HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 Antibody,RPGR-interacting protein 1 Antibody,RPGRIP1 Antibody

More 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)
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Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-RPGRIP1 Antibody

Catalogue No.

CSB-PA850326ESR2HU

Reactivity

Human

Immunogen

Recombinant human X-linked retinitis pigmentosa GTPase regulator-interacting protein 1 protein (400-580AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

146 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:57096( Human), Omim:605446( Human), SwissProt:Q96KN7( Human), Unigene:126035( Human)

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. .

Note

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Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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