Antibody data
- Antibody Data
- Antigen structure
- References [17]
- Comments [0]
- Validations [0]
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Validation data
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- Product number
- HPA001593 - Provider product page
- Provider
- Atlas Antibodies
- Proper citation
- Atlas Antibodies Cat#HPA001593, RRID:AB_1079835
- Product name
- Anti-RPGR
- Antibody type
- Polyclonal
- Description
- Polyclonal Antibody against Human RPGR, Gene description: retinitis pigmentosa GTPase regulator, Alternative Gene Names: COD1, CORDX1, CRD, RP15, RP3, Validated applications: IHC, Uniprot ID: Q92834, Storage: Store at +4°C for short term storage. Long time storage is recommended at -20°C.
- Reactivity
- Human
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 100 µl
- Concentration
- 0.1 mg/ml
- Storage
- Store at +4°C for short term storage. Long time storage is recommended at -20°C.
- Handling
- The antibody solution should be gently mixed before use.
Submitted references Interactome Analysis Reveals a Link of the Novel ALMS1-CEP70 Complex to Centrosomal Clusters
Retinal prolactin isoform PRLΔE1 sustains rod disease in inherited retinal degenerations
Skewed X-inactivation is associated with retinal dystrophy in female carriers ofRPGRmutations
Human iPSC-Derived Retinal Organoids and Retinal Pigment Epithelium for Novel Intronic RPGR Variant Assessment for Therapy Suitability
The Major Ciliary Isoforms of RPGR Build Different Interaction Complexes with INPP5E and RPGRIP1L
In Vivo CRISPR/Cas9-Mediated Genome Editing Mitigates Photoreceptor Degeneration in a Mouse Model of X-Linked Retinitis Pigmentosa
Translational Read-Through Therapy of RPGR Nonsense Mutations
A quantitative super-resolution imaging toolbox for diagnosis of motile ciliopathies
Disruption of RPGR protein interaction network is the common feature of RPGR missense variations that cause XLRP
Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish
Optimization of Retinal Gene Therapy for X-Linked Retinitis Pigmentosa Due to RPGR Mutations
Codon-Optimized RPGR Improves Stability and Efficacy of AAV8 Gene Therapy in Two Mouse Models of X-Linked Retinitis Pigmentosa
RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner
Stability and Safety of an AAV Vector for Treating RPGR-ORF15 X-Linked Retinitis Pigmentosa
Successful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease
The role of RPGR in cilia formation and actin stability
Zebrafish Rpgr is required for normal retinal development and plays a role in dynein-based retrograde transport processes
Woerz F, Hoffmann F, Antony S, Bolz S, Jarboui M, Junger K, Klose F, Stehle I, Boldt K, Ueffing M, Beyer T
Molecular & Cellular Proteomics 2024;23(1):100701
Molecular & Cellular Proteomics 2024;23(1):100701
Retinal prolactin isoform PRLΔE1 sustains rod disease in inherited retinal degenerations
Sudharsan R, Kwok J, Swider M, Sumaroka A, Aguirre G, Cideciyan A, Beltran W
Cell Death & Disease 2024;15(9)
Cell Death & Disease 2024;15(9)
Skewed X-inactivation is associated with retinal dystrophy in female carriers ofRPGRmutations
Usman M, Jüschke C, Song F, Kastrati D, Owczarek-Lipska M, Eilers J, Pauleikhoff L, Lange C, Neidhardt J
Life Science Alliance 2023;6(10):e202201814
Life Science Alliance 2023;6(10):e202201814
Human iPSC-Derived Retinal Organoids and Retinal Pigment Epithelium for Novel Intronic RPGR Variant Assessment for Therapy Suitability
Chahine Karam F, Loi T, Ma A, Nash B, Grigg J, Parekh D, Riley L, Farnsworth E, Bennetts B, Gonzalez-Cordero A, Jamieson R
Journal of Personalized Medicine 2022;12(3):502
Journal of Personalized Medicine 2022;12(3):502
The Major Ciliary Isoforms of RPGR Build Different Interaction Complexes with INPP5E and RPGRIP1L
Vössing C, Atigbire P, Eilers J, Markus F, Stieger K, Song F, Neidhardt J
International Journal of Molecular Sciences 2021;22(7):3583
International Journal of Molecular Sciences 2021;22(7):3583
In Vivo CRISPR/Cas9-Mediated Genome Editing Mitigates Photoreceptor Degeneration in a Mouse Model of X-Linked Retinitis Pigmentosa
Hu S, Du J, Chen N, Jia R, Zhang J, Liu X, Yang L
Investigative Opthalmology & Visual Science 2020;61(4):31
Investigative Opthalmology & Visual Science 2020;61(4):31
Translational Read-Through Therapy of RPGR Nonsense Mutations
Vössing C, Owczarek-Lipska M, Nagel-Wolfrum K, Reiff C, Jüschke C, Neidhardt J
International Journal of Molecular Sciences 2020;21(22):8418
International Journal of Molecular Sciences 2020;21(22):8418
A quantitative super-resolution imaging toolbox for diagnosis of motile ciliopathies
Liu Z, Nguyen Q, Guan Q, Albulescu A, Erdman L, Mahdaviyeh Y, Kang J, Ouyang H, Hegele R, Moraes T, Goldenberg A, Dell S, Mennella V
Science Translational Medicine 2020;12(535)
Science Translational Medicine 2020;12(535)
Disruption of RPGR protein interaction network is the common feature of RPGR missense variations that cause XLRP
Zhang Q, Giacalone J, Searby C, Stone E, Tucker B, Sheffield V
Proceedings of the National Academy of Sciences 2019;116(4):1353-1360
Proceedings of the National Academy of Sciences 2019;116(4):1353-1360
Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish
Raghupathy R, Zhang X, Liu F, Alhasani R, Biswas L, Akhtar S, Pan L, Moens C, Li W, Liu M, Kennedy B, Shu X
Scientific Reports 2017;7(1)
Scientific Reports 2017;7(1)
Optimization of Retinal Gene Therapy for X-Linked Retinitis Pigmentosa Due to RPGR Mutations
Beltran W, Cideciyan A, Boye S, Ye G, Iwabe S, Dufour V, Marinho L, Swider M, Kosyk M, Sha J, Boye S, Peterson J, Witherspoon C, Alexander J, Ying G, Shearman M, Chulay J, Hauswirth W, Gamlin P, Jacobson S, Aguirre G
Molecular Therapy 2017;25(8):1866-1880
Molecular Therapy 2017;25(8):1866-1880
Codon-Optimized RPGR Improves Stability and Efficacy of AAV8 Gene Therapy in Two Mouse Models of X-Linked Retinitis Pigmentosa
Fischer M, McClements M, Martinez-Fernandez de la Camara C, Bellingrath J, Dauletbekov D, Ramsden S, Hickey D, Barnard A, MacLaren R
Molecular Therapy 2017;25(8):1854-1865
Molecular Therapy 2017;25(8):1854-1865
RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner
Dutta N, Seo S
Biology Open 2016;5(9):1283-1289
Biology Open 2016;5(9):1283-1289
Stability and Safety of an AAV Vector for Treating RPGR-ORF15 X-Linked Retinitis Pigmentosa
Deng W, Dyka F, Dinculescu A, Li J, Zhu P, Chiodo V, Boye S, Conlon T, Erger K, Cossette T, Hauswirth W
Human Gene Therapy 2015;26(9):593-602
Human Gene Therapy 2015;26(9):593-602
Successful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease
Beltran W, Cideciyan A, Iwabe S, Swider M, Kosyk M, McDaid K, Martynyuk I, Ying G, Shaffer J, Deng W, Boye S, Lewin A, Hauswirth W, Jacobson S, Aguirre G
Proceedings of the National Academy of Sciences 2015;112(43)
Proceedings of the National Academy of Sciences 2015;112(43)
The role of RPGR in cilia formation and actin stability
Gakovic M, Shu X, Kasioulis I, Carpanini S, Moraga I, Wright A
Human Molecular Genetics 2011;20(24):4840-4850
Human Molecular Genetics 2011;20(24):4840-4850
Zebrafish Rpgr is required for normal retinal development and plays a role in dynein-based retrograde transport processes
Shu X, Zeng Z, Gautier P, Lennon A, Gakovic M, Patton E, Wright A
Human Molecular Genetics 2010;19(4):657-670
Human Molecular Genetics 2010;19(4):657-670
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