Anti RPGR pAb (ATL-HPA001593)

Atlas Antibodies

Catalog No.: ATL-HPA001593-25

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.

Product Specifications

• Application: IHC
• Reactivity: Human
• Clonality: Polyclonal
• Host: Rabbit
• Immunogen: EINDTCLSVATFLPYSSLTSGNVLQRTLSARMRRRERERSPDSFSMRRTLPPIEGTLGLSACFLPNSVFPRCSERNLQESVLSEQDLMQPEEPDYLLDEMTKEAEIDNSSTVESLGETTDILNMTHIMSLN
• Gene Sequence: EINDTCLSVATFLPYSSLTSGNVLQRTLSARMRRRERERSPDSFSMRRTLPPIEGTLGLSACFLPNSVFPRCSERNLQESVLSEQDLMQPEEPDYLLDEMTKEAEIDNSSTVESLGETTDILNMTHIMSLN
• Gene ID - Mouse: ENSMUSG00000031174
• Gene ID - Rat: ENSRNOG00000003013
• Buffer: 40% glycerol and PBS (pH 7.2). 0.02% sodium azide is added as preservative.

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

Deng, Wen-Tao; Dyka, Frank M; Dinculescu, Astra; Li, Jie; Zhu, Ping; Chiodo, Vince A; Boye, Sanford L; Conlon, Thomas J; Erger, Kirsten; Cossette, Travis; Hauswirth, William W. Stability and Safety of an AAV Vector for Treating RPGR-ORF15 X-Linked Retinitis Pigmentosa. Human Gene Therapy. 2015;26(9):593-602. 

Fischer, M Dominik; McClements, Michelle E; Martinez-Fernandez de la Camara, Cristina; Bellingrath, Julia-Sophia; Dauletbekov, Daniyar; Ramsden, Simon C; Hickey, Doron G; Barnard, Alun R; MacLaren, Robert E. Codon-Optimized RPGR Improves Stability and Efficacy of AAV8 Gene Therapy in Two Mouse Models of X-Linked Retinitis Pigmentosa. Molecular Therapy : The Journal Of The American Society Of Gene Therapy. 2017;25(8):1854-1865. 

Beltran, William A; Cideciyan, Artur V; Boye, Shannon E; Ye, Guo-Jie; Iwabe, Simone; Dufour, Valerie L; Marinho, Luis Felipe; Swider, Malgorzata; Kosyk, Mychajlo S; Sha, Jin; Boye, Sanford L; Peterson, James J; Witherspoon, C Douglas; Alexander, John J; Ying, Gui-Shuang; Shearman, Mark S; Chulay, Jeffrey D; Hauswirth, William W; Gamlin, Paul D; Jacobson, Samuel G; Aguirre, Gustavo D. Optimization of Retinal Gene Therapy for X-Linked Retinitis Pigmentosa Due to RPGR Mutations. Molecular Therapy : The Journal Of The American Society Of Gene Therapy. 2017;25(8):1866-1880. 

Raghupathy, Rakesh K; Zhang, Xun; Liu, Fei; Alhasani, Reem H; Biswas, Lincoln; Akhtar, Saeed; Pan, Luyuan; Moens, Cecilia B; Li, Wenchang; Liu, Mugen; Kennedy, Breandan N; Shu, Xinhua. Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish. Scientific Reports. 2017;7(1):16881. 

Zhang, Qihong; Giacalone, Joseph C; Searby, Charles; Stone, Edwin M; Tucker, Budd A; Sheffield, Val C. Disruption of RPGR protein interaction network is the common feature of RPGR missense variations that cause XLRP. Proceedings Of The National Academy Of Sciences Of The United States Of America. 2019;116(4):1353-1360. 

Chahine Karam, Fidelle; Loi, To Ha; Ma, Alan; Nash, Benjamin M; Grigg, John R; Parekh, Darshan; Riley, Lisa G; Farnsworth, Elizabeth; Bennetts, Bruce; Gonzalez-Cordero, Anai; Jamieson, Robyn V. Human iPSC-Derived Retinal Organoids and Retinal Pigment Epithelium for Novel Intronic RPGR Variant Assessment for Therapy Suitability. Journal Of Personalized Medicine. 2022;12(3) 

Shu, Xinhua; Zeng, Zhiqiang; Gautier, Philippe; Lennon, Alan; Gakovic, Milica; Patton, E Elizabeth; Wright, Alan F. Zebrafish Rpgr is required for normal retinal development and plays a role in dynein-based retrograde transport processes. Human Molecular Genetics. 2010;19(4):657-70. 

Gakovic, Milica; Shu, Xinhua; Kasioulis, Ioannis; Carpanini, Sarah; Moraga, Ignacio; Wright, Alan F. The role of RPGR in cilia formation and actin stability. Human Molecular Genetics. 2011;20(24):4840-50. 

Beltran, William A; Cideciyan, Artur V; Iwabe, Simone; Swider, Malgorzata; Kosyk, Mychajlo S; McDaid, Kendra; Martynyuk, Inna; Ying, Gui-Shuang; Shaffer, James; Deng, Wen-Tao; Boye, Sanford L; Lewin, Alfred S; Hauswirth, William W; Jacobson, Samuel G; Aguirre, Gustavo D. Successful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease. Proceedings Of The National Academy Of Sciences Of The United States Of America. 2015;112(43):E5844-53. 

Dutta, Nirmal; Seo, Seongjin. RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner. Biology Open. 2016;5(9):1283-9. 

Megaw, Roly; Abu-Arafeh, Hashem; Jungnickel, Melissa; Mellough, Carla; Gurniak, Christine; Witke, Walter; Zhang, Wei; Khanna, Hemant; Mill, Pleasantine; Dhillon, Baljean; Wright, Alan F; Lako, Majlinda; Ffrench-Constant, Charles. Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models. Nature Communications. 2017;8(1):271. 

Liu, Zhen; Nguyen, Quynh P H; Guan, Qingxu; Albulescu, Alexandra; Erdman, Lauren; Mahdaviyeh, Yasaman; Kang, Jasmine; Ouyang, Hong; Hegele, Richard G; Moraes, Theo; Goldenberg, Anna; Dell, Sharon D; Mennella, Vito. A quantitative super-resolution imaging toolbox for diagnosis of motile ciliopathies. Science Translational Medicine. 2020;12(535) 

Hu, Shuang; Du, Juan; Chen, Ningning; Jia, Ruixuan; Zhang, Jinlu; Liu, Xiaozhen; Yang, Liping. In Vivo CRISPR/Cas9-Mediated Genome Editing Mitigates Photoreceptor Degeneration in a Mouse Model of X-Linked Retinitis Pigmentosa. Investigative Ophthalmology & Visual Science. 2020;61(4):31. 

Vössing, Christine; Owczarek-Lipska, Marta; Nagel-Wolfrum, Kerstin; Reiff, Charlotte; Jüschke, Christoph; Neidhardt, John. Translational Read-Through Therapy of RPGR Nonsense Mutations. International Journal Of Molecular Sciences. 2020;21(22)