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The antibody against RPGRIP1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1091-1286 of human RPGRIP1 (NP_065099.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IF/ICC, ELISA.
The antibody against RPGRIP1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1091-1286 of human RPGRIP1 (NP_065099.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on IF/ICC, ELISA.
Perform high pressure antigen retrieval with 10 mM citrate buffer pH 6.0 before commencing with IF staining protocol.Immunofluorescence analysis of mouse eye cells using RPGRIP1 Rabbit pAb at dilution of 1:200 (40x lens). Blue: DAPI for nuclear staining.
| Cat.No | ADA-08202A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | RPGRIP1 |
| Target Synonyms | RPGRIP1; CORD13; LCA6; RGI1; RGRIP; RPGRIP; RPGRIP1d; retinitis pigmentosa GTPase regulator interacting protein 1 | Form | Liquid |
| Species Reactivity | Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.05% proclin300, pH7.3. | Purification Method | Affinity purification |
| Application | ELISA, IF/ICC |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1091-1286 of human RPGRIP1 (NP_065099.3). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | SEAQTTDSDDVIVPPMSQKYPKADSEKMCIEIVSLAFYPEAEVMSDENIKQVYVEYKFYDLPLSETETPVSLRKPRAGEEIHFHFSKVIDLDPQEQQGRRRFLFDMLNGQDPDQGHLKFTVVSDPLDEEKKECEEVGYAYLQLWQILESGRDILEQELDIVSPEDLATPIGRLKVSLQAAAVLHAIYKEMTEDLFS | Uniprot ID | Q96KN7 |
Uniprot Id
Q96KN7
Target Species
Human
Target Name
RPGRIP1
Target Full Name
X-linked retinitis pigmentosa GTPase regulator-interacting protein 1
Target 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.
Target Involvement
Leber congenital amaurosis 6 (LCA6); Cone-rod dystrophy 13 (CORD13)
Target Subcellular Location
Cell projection, cilium.
Target Protein Families
RPGRIP1 family
Target 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.
Target Synonyms
RPGRIP1; X-linked retinitis pigmentosa GTPase regulator-interacting protein 1; RPGR-interacting protein 1
Target Background
This gene encodes a photoreceptor protein that interacts with retinitis pigmentosa GTPase regulator protein and is a key component of cone and rod photoreceptor cells. Mutations in this gene lead to autosomal recessive congenital blindness.
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