RP2 Antibody (PACO44414)
- SKU:
- PACO44414
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- IHC
- IF
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Frequently bought together:
Description
| Antibody Name: | RP2 Antibody (PACO44414) |
| Antibody SKU: | PACO44414 |
| Size: | 50ul |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC, IF |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200, IF:1:50-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human Protein XRP2 protein (1-350AA) |
| Form: | Liquid |
| Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Purification Method: | Antigen Affinity Purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Immunohistochemistry of paraffin-embedded human kidney tissue using PACO44414 at dilution of 1:100. |
 | Immunofluorescent analysis of Hela cells using PACO44414 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
| Background: | Acts as a GTPase-activating protein (GAP) involved in trafficking between the Golgi and the ciliary membrane. Involved in localization of proteins, such as NPHP3, to the cilium membrane by inducing hydrolysis of GTP ARL3, leading to the release of UNC119 (or UNC119B). Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. Acts as guanine nucleotide dissociation inhibitor towards ADP-ribosylation factor-like proteins. |
| Synonyms: | Protein XRP2, RP2 |
| UniProt Protein Function: | RP2: Acts as a GTPase-activating protein (GAP) involved in trafficking between the Golgi and the ciliary membrane. Involved in localization of proteins, such as NPHP3, to the cilium membrane by inducing hydrolysis of GTP ARL3, leading to the release of UNC119 (or UNC119B). Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. Acts as guanine nucleotide dissociation inhibitor towards ADP-ribosylation factor-like proteins. Defects in RP2 are the cause of retinitis pigmentosa type 2 (RP2); also known as X-linked retinitis pigmentosa 2 (XLRP-2). 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. Belongs to the TBCC family.Protein type: ChaperoneChromosomal Location of Human Ortholog: Xp11.3Cellular Component: centriole; Golgi apparatus; cytoplasm; plasma membrane; cytoplasmic vesicleMolecular Function: protein binding; GTP binding; unfolded protein binding; nucleoside diphosphate kinase activity; actin binding; GTPase activator activity; ATP bindingBiological Process: GTP biosynthetic process; CTP biosynthetic process; protein transport; protein folding; visual perception; organelle organization and biogenesis; UTP biosynthetic process; cell morphogenesis; nucleoside diphosphate phosphorylation; cytoskeleton organization and biogenesis; post-Golgi vesicle-mediated transport; post-chaperonin tubulin folding pathway; positive regulation of GTPase activityDisease: Retinitis Pigmentosa 2 |
| UniProt Protein Details: | |
| NCBI Summary: | The RP2 locus has been implicated as one cause of X-linked retinitis pigmentosa. The predicted gene product shows homology with human cofactor C, a protein involved in the ultimate step of beta-tubulin folding. Progressive retinal degeneration may therefore be due to the accumulation of incorrectly-folded photoreceptor or neuron-specific tubulin isoforms followed by progressive cell death [provided by RefSeq, Jul 2008] |
| UniProt Code: | O75695 |
| NCBI GenInfo Identifier: | 60416394 |
| NCBI Gene ID: | 6102 |
| NCBI Accession: | O75695.4 |
| UniProt Secondary Accession: | O75695 |
| UniProt Related Accession: | O75695 |
| Molecular Weight: | |
| NCBI Full Name: | Protein XRP2 |
| NCBI Synonym Full Names: | RP2 activator of ARL3 GTPase |
| NCBI Official Symbol: | RP2Â Â |
| NCBI Official Synonym Symbols: | XRP2; NME10; TBCCD2; NM23-H10; DELXp11.3Â Â |
| NCBI Protein Information: | protein XRP2 |
| UniProt Protein Name: | Protein XRP2 |
| UniProt Synonym Protein Names: | |
| Protein Family: | Protein |
| UniProt Gene Name: | RP2Â Â |
| UniProt Entry Name: | XRP2_HUMAN |
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