|Application ||WB, IHC-P, E|
|Dilution||ELISA (1:20000), IHC-P (10 µg/ml), WB (1:500-1:1000)|
|Other Names||Centrosomal protein of 290 kDa, Cep290, Bardet-Biedl syndrome 14 protein, Cancer/testis antigen 87, CT87, Nephrocystin-6, Tumor antigen se2-2, CEP290, BBS14, KIAA0373, NPHP6|
|Target/Specificity||CEP290 Antibody detects endogenous levels of total CEP290 protein.|
|Reconstitution & Storage||Store at -20°C for up to one year.|
|Precautions||CEP290 Antibody (aa771-820) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Synonyms||BBS14, KIAA0373, NPHP6|
|Function||Part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition (By similarity). Activates ATF4-mediated transcription. Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes.|
|Cellular Location||Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite Nucleus. Cell projection, cilium. Cytoplasm, cytoskeleton, cilium basal body. Note=Connecting cilium of photoreceptor cells, base of cilium in kidney intramedullary collecting duct cells. Localizes at the transition zone, a region between the basal body and the ciliary axoneme (By similarity). Displaced from centriolar satellites in response to cellular stress, such as ultraviolet light (UV) radiation or heat shock.|
|Tissue Location||Ubiquitous. Expressed strongly in placenta and weakly in brain.|
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Provided below are standard protocols that you may find useful for product applications.
Part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition (By similarity). Activates ATF4-mediated transcription. Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes.
Sayer J.A.,et al.Nat. Genet. 38:674-681(2006).
Nagase T.,et al.DNA Res. 4:141-150(1997).
Scherer S.E.,et al.Nature 440:346-351(2006).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Eichmueller S.,et al.Proc. Natl. Acad. Sci. U.S.A. 98:629-634(2001).
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