CEP290 Rabbit Polyclonal Antibody

CAT#: AP21146PU-N

CEP290 rabbit polyclonal antibody, Aff - Purified

Product Images

Western blot (WB) analysis of CEP290 antibody (Cat.-No.: AP21146PU-N) in extracts from K562 cells.

Specifications

Product Data

• Applications: WB
• Recommended Dilution: Western Blot: 1/500-1/1000.
• Reactivities: 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: Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer.
  Avoid repeated freezing and thawing.
• Stability: Shelf life: one year from despatch.
• 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