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Retinitis Pigmentosa GTPase Regulator (RPGR) ELISA Kits

RPGR encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. Additionally we are shipping RPGR Antibodies (33) and RPGR Proteins (3) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
Anti-Rat RPGR RPGR 367733  
RPGR 6103 Q92834
RPGR 19893 Q9R0X5
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Top RPGR ELISA Kits at antibodies-online.com

Showing 3 out of 3 products:

Catalog No. Reactivity Sensitivity Range Quantity Supplier Delivery Price Details
Human
96 Tests Log in to see 11 to 13 Days
$875.60
Details
Mouse
96 Tests Log in to see 11 to 13 Days
$875.60
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Dog
96 Tests Log in to see 11 to 13 Days
$1,095.60
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More ELISA Kits for RPGR Interaction Partners

Human Retinitis Pigmentosa GTPase Regulator (RPGR) interaction partners

  1. Profound visual loss occurred by the second decade of life with progression to near no light perception by age 60 in this kindred of X-linked RP associated with the RPGR genotype.

  2. The regulator of chromosome condensation 1-like (show RCBTB2 ELISA Kits) domain of RPGR was conserved in vertebrates and invertebrates, but RPGR(ORF15) was unique to vertebrates.

  3. Coverage-based analysis indicated that the RPGR open reading frame (ORF)15 was located in an uncovered or low-depth region. Through additional screening of ORF15, we identified pathogenic mutations in 14% (7/50) of patients.

  4. X-linked retinitis pigmentosa caused by mutations in the RPGR gene is a severe and early onset form of retinal degeneration. [review]

  5. Severe retinal degeneration is found in a Czech family women with a c.2543del mutation in ORF15 of the RPGR gene.

  6. RPGR mutations associated with X-linked retinitis pigmentosa.

  7. We will summarize the functional characterization of RPGR and highlight recent studies in animal models, which will not only shed light on the disease mechanisms in X linked retinitis pigmentosa but will also provide therapeutic strategies for treatment.

  8. A novel RPGR gene was found in a retinal dystrophy (show MERTK ELISA Kits) patient in a family with Stargardt disease.

  9. RPGR is acting as a scaffold protein recruiting cargo-loaded PDE6D and Arl3 to release lipidated cargo into cilia.

  10. Mutations in RPGR were found in two patients and relatives with primary ciliary dyskinesia and retinitis pigmentosa. Reduced ciliary orientation and coordination of ciliary bundles suggest RPGR may play a role in respiratory cilia orientation.

Mouse (Murine) Retinitis Pigmentosa GTPase Regulator (RPGR) interaction partners

  1. Given a frequent occurrence of RPGR mutations in severe photoreceptor degeneration due to ciliary disorders, our results provide insights into pathways resulting in altered mature cilia function in ciliopathies.

  2. Data shsow that Rpgr ORF15 transcripts, but not protein, were detected in retinas from Rd9 (show PITPNM1 ELISA Kits)/Y male mice that exhibited retinal pathology.

  3. Misexpression of Rpgr(ex1 (show FRMD6 ELISA Kits)-19) causes retinal degeneration that is considerably more severe than that caused by Rpgr knockout but photoreceptors tolerate overexpression of Rpgr(ORF15) without evidence of degeneration.

  4. RPGR localizes to the podocytes in the glomerulus as well as to primary cilia in parietal epithelium and tubules

  5. RPGR and RPGRIP (show RPGRIP1 ELISA Kits) isoforms are distributed and co-localized at restricted foci throughout the outer segments of human and bovine, but not mice rod photoreceptors.

  6. Certain truncated forms of RPGR can behave as a dominant, gain-of-function mutant.

  7. a CEP290/NPHP6 (show CEP290 ELISA Kits) mutation perturbs its interaction with RPGR and results in early-onset retinal degeneration

  8. This complexity of defects in flagellar assembly suggests a role of RPGR in intraflagellar transport processes.

RPGR Antigen Profile

Antigen Summary

This gene encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. The encoded protein is found in the Golgi body and interacts with RPGRIP1. This protein localizes to the outer segment of rod photoreceptors and is essential for their viability. Mutations in this gene have been associated with X-linked retinitis pigmentosa (XLRP). Multiple alternatively spliced transcript variants that encode different isoforms of this gene have been reported, but the full-length natures of only some have been determined.

Gene names and symbols associated with RPGR

  • retinitis pigmentosa GTPase regulator (Rpgr) antibody
  • retinitis pigmentosa GTPase regulator (RPGR) antibody
  • retinitis pigmentosa GTPase regulator (rpgr) antibody
  • retinitis pigmentosa GTPase regulator (Bm1_19935) antibody
  • retinitis pigmentosa GTPase regulator (NAEGRDRAFT_80250) antibody
  • COD1 antibody
  • CORDX1 antibody
  • CRD antibody
  • orf15 antibody
  • PCDX antibody
  • RP3 antibody
  • Rp3h antibody
  • RP15 antibody
  • XLRP3 antibody
  • xrpgr antibody

Protein level used designations for RPGR

X-linked retinitis pigmentosa GTPase regulator , retinitis pigmentosa GTPase regulator , RPGR 1-19 isoform , X-linked retinitis pigmentosa GTPase regulator-like , retinitis pigmentosa 15 , retinitis pigmentosa 3 GTPase regulator , mRpgr , retinitis pigmentosa GTP-ase regulator RPGR

GENE ID SPECIES
367733 Rattus norvegicus
418578 Gallus gallus
513263 Bos taurus
554332 Ovis aries
698053 Macaca mulatta
733454 Xenopus (Silurana) tropicalis
734172 Xenopus laevis
6098892 Brugia malayi
8852870 Naegleria gruberi strain NEG-M
100170141 Sus scrofa
100389535 Callithrix jacchus
6103 Homo sapiens
19893 Mus musculus
403726 Canis lupus familiaris
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