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, No Gclids
RPGR Antibodies
AND RPGR ELISA kits, RPGR Proteins
Many biological assays use antibodies to detect RPGR, such as Western Blot (WB), ELISA, Flow Cytometry, Immunocytochemistry (IHC). These antibodies can be polyclonal or monoclonal, and react with RPGR in Human, Mouse, Rat, and many other animal samples. Boster Bio use mainly mouse and rabbit to develop RPGR antibodies...
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RPGR Infographic by Boster Bio
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RPGR Overview
Facts about X-linked retinitis pigmentosa GTPase regulator.
X-linked retinitis pigmentosa GTPase regulator (RPGR)
Could be a guanine-nucleotide releasing factor. Plays a role in ciliogenesis.
Probably regulates cilia formation by regulating actin stress filaments and cell contractility. Plays an important role in photoreceptor integrity.
Gene Information
| Human | |
|---|---|
| Gene Name: | RPGR |
| Uniprot: | Q92834 |
| Entrez: | 6103 |
Family
| Belongs to: |
|---|
| No superfamily |
Alternative Names
COD1; cone dystrophy 1 (X-linked); CORDX1RP3retinitis pigmentosa 15; CRD; orf15; PCDX; retinitis pigmentosa 3 GTPase regulator; retinitis pigmentosa GTPase regulator; RP15; X-linked retinitis pigmentosa GTPase regulator; XLRP3
Molecular Weight
Mass (kDA):
113.387 kDA
Genomic Context
| Human | |
|---|---|
| Location: | Xp11.4 |
| Sequence: | X; NC_000023.11 (38269163..38327509, complement) |
Tissue Specificity
Heart, brain, placenta, lung, liver, muscle, kidney, retina, pancreas and fetal retinal pigment epithelium. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments.
Subcellular Localizations
Cytoplasm, cytoskeleton, flagellum axoneme. Golgi apparatus. Cell projection, cilium. In the retinal photoreceptor cell layer, localizes at the connecting cilium (By similarity). Colocalizes with WHRN in the photoreceptor connecting cilium (By similarity). Colocalizes with CEP290 in the photoreceptor connecting cilium (By similarity). Colocalizes with RPGRIP1 in the photoreceptor connecting cilium (By similarity).; [Isoform 6]: Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton, cilium axoneme.
Diseases
- Retinitis Pigmentosa
- Genetic Diseases, X-linked
- Retinal Degeneration
- Retinal Diseases
- Dystrophy
- Blind Vision
- Retinal Dystrophies
- Leber Congenital Amaurosis
- Amaurosis
- Atrophy
- Myopia
Interacting Proteins
- NPHP4
- PDE6D
- Pde6d
- RPGRIP1
Pathways
- Localization
- Pathogenesis
- Transport
- Reflex
- Translation
- Cell Death
- Phototransduction
- Intraflagellar Transport
- Chromosome Condensation
- Proteolysis
- Urea Cycle
- Cell Cycle
- Intracellular Transport
PTMs
- Prenylation
For details, visit:
bosterbio.com/bosterbio-gene-info-cards/RPGR
bosterbio.com/bosterbio-gene-info-cards/RPGR
References
PMID: 8673101 by Meindl A., et al. A gene (RPGR) with homology to the RCC1 guanine nucleotide exchange factor is mutated in X-linked retinitis pigmentosa (RP3).
PMID: 8817343 by Roepman R., et al. Positional cloning of the gene for X-linked retinitis pigmentosa 3: homology with the guanine-nucleotide-exchange factor RCC1.