Anti-RPGR Polyclonal Antibody for WB, IF-P, IHC-P, ELISA - Q92834

Core Product Specifications and Parameters

Biological Background: RPGR Function and Localization

• Protein identity: RPGR (X-linked retinitis pigmentosa GTPase regulator) is also known by aliases CRD, RP3, COD1, PCDX, RP15, XLRP3, orf15, and CORDX1. Multiple isoforms arise from alternative splicing, with isoform 3 being retina-specific.
• GEF activity and ciliary trafficking: RPGR acts as a guanine-nucleotide releasing factor (GEF) for RAB8A and RAB37, converting inactive GDP-bound RABs to active GTP-bound forms. This GEF activity on RAB8A facilitates ciliary trafficking and is crucial for photoreceptor integrity. Related references: PMID:20631154
• Cilia formation and organization: RPGR regulates actin stress filaments and cell contractility, and is involved in microtubule organization. It plays a role in primary cilia formation and intraflagellar transport. Related references: PMID:21933838
• Subcellular localization: RPGR localizes to the cytoplasm, Golgi apparatus, ciliary basal body, axoneme, centrosome, and flagellum axoneme, consistent with its role in ciliary and microtubule-related processes.
• Tissue specificity: Ubiquitously expressed, with higher levels in heart, brain, placenta, lung, liver, muscle, kidney, retina, and pancreas. Retina-specific isoform 3 colocalizes with RPGRIP1 in the outer segment of rod and cone photoreceptors.
• Disease associations: Mutations in RPGR are linked to X-linked retinitis pigmentosa, cone-rod dystrophy, and ciliopathies, underscoring its essential role in vision and sensory signal transduction.
• Post-translational modifications: RPGR is prenylated, methylated, and phosphorylated, and it is classified as a lipoprotein. These modifications likely influence its membrane anchoring and protein interactions.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the N-terminal region (aa 1–260) of human RPGR. The antibody may detect multiple isoforms; validate specificity in your target sample system (e.g., retinal extracts).
• In Western blot (WB), expect a band near the predicted full-length molecular weight of ~113 kDa. Use appropriate gel percentage and transfer conditions for high-molecular-weight proteins.
• For immunofluorescence (IF-P) and immunohistochemistry (IHC-P), RPGR localizes to cilia and centrioles. Optimize fixation and permeabilization protocols to preserve delicate ciliary structures.
• Reported cross-reactivity with mouse and rat suggests epitope conservation; align sequences if using non-human models. Colocalization with RAB8A or ciliary markers (e.g., acetylated tubulin) may be informative.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers and research institutes with a one-stop supply of IVD raw materials, from validated antibody pairs to bulk ancillary reagents. Our RPGR polyclonal antibody supports cutting-edge vision and ciliopathy research, with reliable performance across multiple applications. Contact us for tailored sourcing solutions, technical consultation, and seamless integration of this reagent into your assay development pipeline.