Anti RPGR pAb (ATL-HPA001593)

Atlas

Catalog No: ATL-HPA001593-25

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Application: IHC 
Clonality: Polyclonal 
Host: Rabbit 
Reactivity: Human 
Protein Description: retinitis pigmentosa GTPase regulator
Gene Name: RPGR
Alternative Gene Name: COD1, CORDX1, CRD, RP15, RP3
Isotype: IgG
Interspecies mouse/rat: ENSMUSG00000031174: 46%, ENSRNOG00000003013: 41%
Entrez Gene ID: 6103
Uniprot ID: Q92834
Buffer: 40% glycerol and PBS (pH 7.2). 0.02% sodium azide is added as preservative.
Storage Temperature: Store at +4°C for short term storage. Long time storage is recommended at -20°C.

Citations for Anti RPGR pAb (ATL-HPA001593) – 14 Found

Stability and Safety of an AAV Vector for Treating RPGR-ORF15 X-Linked Retinitis Pigmentosa.
Hum Gene Ther. 2015 Sep;26(9):593-602. doi: 10.1089/hum.2015.035. Epub 2015 Jul 29. 

Codon-Optimized RPGR Improves Stability and Efficacy of AAV8 Gene Therapy in Two Mouse Models of X-Linked Retinitis Pigmentosa.
Mol Ther. 2017 Aug 2;25(8):1854-1865. doi: 10.1016/j.ymthe.2017.05.005. Epub 2017 May 24. 

Optimization of Retinal Gene Therapy for X-Linked Retinitis Pigmentosa Due to RPGR Mutations.
Mol Ther. 2017 Aug 2;25(8):1866-1880. doi: 10.1016/j.ymthe.2017.05.004. Epub 2017 May 27. 

Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish.
Sci Rep. 2017 Dec 4;7(1):16881. doi: 10.1038/s41598-017-12838-x. 

Disruption of RPGR protein interaction network is the common feature of RPGR missense variations that cause XLRP.
Proc Natl Acad Sci U S A. 2019 Jan 22;116(4):1353-1360. doi: 10.1073/pnas.1817639116. Epub 2019 Jan 8. 

Human iPSC-Derived Retinal Organoids and Retinal Pigment Epithelium for Novel Intronic RPGR Variant Assessment for Therapy Suitability.
J Pers Med. 2022 Mar 21;12(3):502. doi: 10.3390/jpm12030502. 

Zebrafish Rpgr is required for normal retinal development and plays a role in dynein-based retrograde transport processes.
Hum Mol Genet. 2010 Feb 15;19(4):657-70. doi: 10.1093/hmg/ddp533. Epub 2009 Dec 1. 

The role of RPGR in cilia formation and actin stability.
Hum Mol Genet. 2011 Dec 15;20(24):4840-50. doi: 10.1093/hmg/ddr423. Epub 2011 Sep 20. 

Successful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease.
Proc Natl Acad Sci U S A. 2015 Oct 27;112(43):E5844-53. doi: 10.1073/pnas.1509914112. Epub 2015 Oct 12. 

RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner.
Biol Open. 2016 Sep 15;5(9):1283-9. doi: 10.1242/bio.020461. 

Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models.
Nat Commun. 2017 Aug 16;8(1):271. doi: 10.1038/s41467-017-00111-8. 

A quantitative super-resolution imaging toolbox for diagnosis of motile ciliopathies.
Sci Transl Med. 2020 Mar 18;12(535):eaay0071. doi: 10.1126/scitranslmed.aay0071. 

In Vivo CRISPR/Cas9-Mediated Genome Editing Mitigates Photoreceptor Degeneration in a Mouse Model of X-Linked Retinitis Pigmentosa.
Invest Ophthalmol Vis Sci. 2020 Apr 9;61(4):31. doi: 10.1167/iovs.61.4.31. 

Translational Read-Through Therapy of RPGR Nonsense Mutations.
Int J Mol Sci. 2020 Nov 10;21(22):8418. doi: 10.3390/ijms21228418.