CEP290 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	Western Blot: 1/500-1/1000.
Reactivity	Human, Mouse
Host	Rabbit
Clonality	Polyclonal
Specificity	This antibody detects endogenous levels of CEP290 protein. (region surrounding Phe812)
Formulation	Phosphate buffered saline (PBS), pH~7.2 State: Aff - Purified State: Liquid purified Ig fraction (> 95% pure by SDS-PAGE). Preservative: 0.05% Sodium Azide
Concentration	1.0 mg/ml
Purification	Affinity Chromatography using epitope-specific immunogen.
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Predicted Protein Size	~ 290 kDa
Gene Name	centrosomal protein 290
Database Link	Entrez Gene 216274 MouseEntrez Gene 80184 Human UniProt ID O15078
Background	CEP290 activates ATF4 mediated transcription and is required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes. CEP290 is ubiquitously expressed; strongly in placenta and weakly in brain. There are two named isoforms. Defects in CEP290 are a cause of Joubert syndrome type 5 (JBTS5) [MIM:610188], Senior-Loken syndrome type 6 (SLSN6) [MIM:610189], Leber congenital amaurosis type 10 (LCA10) [MIM:611755] and Meckel syndrome type 4 (MKS4) [MIM:611134]. Antibodies against CEP290 are present in sera from patients with cutaneous T cell lymphomas, but not in the healthy control population.
Synonyms	Centrosomal protein of 290 kDa, Nephrocystin-6, KIAA0373, NPHP6, se2-2, CT87
Reference Data

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