|Application ||ICC, E|
|Other Accession||NP_079390.3, 80184, 216274 (mouse)|
|Calculated MW||290386 Da|
|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|
|Format||0.5 mg/ml in Tris saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||CEP290 Antibody (internal region) 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.
Despite similarity of the immunizing peptide with the mouse protein, this antibody was demonstrated to weak for mouse Cep290.
Serotype-dependent packaging of large genes in adeno-associated viral vectors results in effective gene delivery in mice. Allocca M, Doria M, Petrillo M, Colella P, Garcia-Hoyos M, Gibbs D, Kim SR, Maguire A, Rex TS, Di Vicino U, Cutillo L, Sparrow JR, Williams DS, Bennett J, Auricchio A. J. Clin. Invest. 2008 May 118 (5): 1955-64. PMID: 18414684
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