Anti-RPGR Rabbit Monoclonal Antibody for WB, IF, ELISA - Q92834-6

Core Product Specifications and Parameters

Biological Background: RPGR Function and Localization

• RPGR (Retinitis Pigmentosa GTPase Regulator) is encoded by the RPGR gene located on the X chromosome, and mutations in this gene are a leading cause of X-linked retinitis pigmentosa (XLRP), characterized by progressive retinal degeneration.
• Isoform 6 is a specific splice variant of RPGR with a predicted molecular weight of approximately 127 kDa; multiple other isoforms exist due to alternative splicing, resulting in proteins of various sizes.
• The protein product of RPGR is classified as a GTPase regulator, suggesting involvement in the regulation of small GTPases, though the exact enzymatic activity and substrates remain under investigation.
• Subcellular localization studies indicate that RPGR isoform 6 is found in the cytoplasm and specifically associates with the cytoskeleton, centrosome, cilium basal body, and cilium axoneme, pointing to a role in ciliary function and maintenance.
• The presence of RPGR at the centrosome and ciliary structures aligns with the clinical manifestation of retinal degeneration, as photoreceptor cells rely heavily on ciliary transport between the inner and outer segments.

Experimental Guidance and Technical Tips

• The immunogen used to raise this antibody spans amino acids 1-260 of human RPGR (isoform 6). Depending on epitope conservation, it may detect other RPGR isoforms; validate cross-reactivity in your specific experimental system.
• Western blot may reveal multiple bands corresponding to the various RPGR isoforms (predicted molecular weights: 52, 70, 83, 90, 113, 127 kDa). Load adequate protein and use isoform-specific controls if precise identification is required.
• For immunofluorescence (IF/ICC, IF-P), expect staining patterns consistent with centrosomal and ciliary localization. Optimize fixation and permeabilization to preserve these structures; consider co-staining with established centrosome or cilia markers.
• This antibody is applicable in ELISA; titrate the antibody and use recombinant RPGR protein as a standard to establish a quantitative detection range.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio empowers diagnostic developers and life science researchers with streamlined access to premium IVD raw materials, exemplified by this RPGR Rabbit mAb. Whether you are advancing retinal disease diagnostics or exploring ciliary biology, our portfolio extends to validated antibody pairs, bulk ancillary reagents, and tailored sourcing for hard-to-find targets. Let CamelBio bridge your concept-to-clinic journey with reliable supply and technical support.