Anti-RPGRIP1 Rabbit Polyclonal Antibody for IF-P and ELISA - Q96KN7

Core Product Specifications and Parameters

Biological Background: RPGRIP1 Function and Localization

• RPGRIP1 is a scaffolding protein required for the proper localization of RPGR at the connecting cilium of photoreceptor cells, essential for normal disk morphogenesis and photoreceptor survival.
• Encoded by the RPGRIP1 gene in humans, it undergoes alternative splicing, producing multiple isoforms with molecular weights ranging from 66 kDa to 146 kDa.
• It is predominantly expressed in the retina, with strong localization in photoreceptor outer segments, and weaker expression in testis and amacrine cells.
• Subcellularly, RPGRIP1 localizes to the cilium and cell projections, specifically at the connecting cilium, where it interacts with RPGR.
• RPGRIP1 contains coiled-coil domains and is associated with the sensory transduction and vision pathways.
• Mutations in RPGRIP1 are linked to inherited retinal disorders, including Leber congenital amaurosis (LCA), cone-rod dystrophy, and other ciliopathies such as glaucoma.
• The protein plays a critical role in photoreceptor disk organization, maintaining the structural integrity of the outer segment.

Experimental Guidance and Technical Tips

• For immunofluorescence (IF-P), consider using retinal sections or cultured photoreceptor cells, and validate the staining pattern in the relevant sample system.
• In ELISA applications, consider pairing with a matched detection antibody and validate with appropriate positive and negative controls to ensure specificity.
• Cross-reactivity with mouse and rat allows the use of rodent models, but always verify reactivity in your specific experimental context.
• Given the multiple isoforms, consider confirming which isoforms are detected in your sample via western blot or other orthogonal methods.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers and research institutes with one-stop access to IVD raw materials, including validated antibodies for retinal disease targets like RPGRIP1. Our portfolio includes optimized polyclonal antibodies, bulk ancillary reagents, and sourcing support for rare targets to accelerate your assay development from concept to clinic.