Anti-NPHP1 Antibody

This rabbit polyclonal antibody targets nephrocystin-1, the protein encoded by the NPHP1 gene. NPHP1 localizes to the transition zone of the primary cilium and the basal body, where it forms part of the NPHP protein module that organizes ciliary membrane composition and gates protein trafficking into and out of cilia. Loss-of-function variants in NPHP1 cause autosomal recessive nephronophthisis type 1 and Joubert syndrome type 4, and the protein is also implicated in Senior-Løken syndrome, a ciliopathy that combines renal cystic disease with retinal degeneration.

Provided by Antibodies.com in a 50 µL unconjugated format, the reagent is raised in rabbit and affinity-purified for use in Western blot and immunofluorescence workflows. The antibody has been validated on samples from human, mouse, and rat, supporting studies of renal epithelial polarity, photoreceptor development, and broader ciliogenesis research. For immunofluorescence, it produces the characteristic basal body and centrosomal signal expected for transition-zone proteins.

Recommended handling: store at -20 °C in small aliquots to limit freeze-thaw cycles, and protect from light to preserve the unconjugated preparation. The polyclonal format provides recognition of multiple epitopes across nephrocystin-1, which is useful when probing patient-derived fibroblasts, kidney biopsy material, or knockdown cell models where a single monoclonal epitope may be lost. Researchers studying Bardet-Biedl syndrome, Joubert syndrome, and related ciliopathies frequently pair NPHP1 staining with markers such as CEP290, RPGRIP1L, and acetylated α-tubulin to map the ciliary compartment.