CEP290 Rabbit Polyclonal Antibody for WB, ELISA - O15078

Core Product Specifications and Parameters

Biological Background: CEP290 Function and Localization

• CEP290 (centrosomal protein of 290 kDa), also known as CT87, MKS4, POC3, BBS14, JBTS5, LCA10, NPHP6, SLSN6, and 3H11Ag, is a large coiled-coil protein involved in early and late steps of cilia formation.
• Its interaction with CCP110 is required for the inhibition of primary cilia formation by CCP110. Related references: PMID:18694559
• CEP290 is required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins such as PCM1 to centrosomes. Related references: PMID:24421332
• It also facilitates efficient recruitment of RAB8A to the primary cilium. Related references: PMID:17705300
• CEP290 regulates the integrity of the BBSome complex and the ciliary targeting of BBSome cargos, possibly involving IQCB1/NPHP5. Related references: PMID:25552655
• Acts as an activator of ATF4-mediated transcription. Related references: PMID:16682973
• Subcellularly, CEP290 localizes to the centrosome, centriolar satellites, ciliary basal body, cilium (including the transition zone as part of the tectonic-like complex), cytoplasmic vesicles, and nucleus.
• It is ubiquitously expressed, with strong expression in placenta and weak in brain, and its mutations are linked to various ciliopathies including Bardet-Biedl syndrome, Joubert syndrome, Leber congenital amaurosis, Meckel syndrome, nephronophthisis, and Senior-Loken syndrome.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 950-1050 of human CEP290 (NP_079390.3). For Western blotting, consider using a low-percentage gel (e.g., 4-12% gradient) to resolve the ~290 kDa protein and include appropriate molecular weight markers.
• Validate antibody specificity in your system by using siRNA-mediated knockdown or CRISPR knockout controls, as CEP290 may have multiple isoforms.
• For ELISA, this polyclonal antibody can be used as capture or detection reagent; optimize coating and detection conditions empirically.
• Due to cross-reactivity limited to human samples, verify reactivity in other species if needed.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio is your one-stop partner for IVD raw materials, offering validated antibody pairs, optimized monoclonal/polyclonal antibodies, and bulk ancillary reagents for diagnostic development. This CEP290 antibody supports research into ciliopathies and centrosome biology, and we can source rare-target raw materials and provide technical consulting to accelerate your projects from concept to clinic.