CEP290 Rabbit Polyclonal Antibody

SKU
AP21146PU-N
CEP290 rabbit polyclonal antibody, Aff - Purified
  $495.00
7 Weeks*
Bulk/Customize
Specifications
Specifications
Product Data
Application WB
Recommended Dilution Western Blot: 1/500-1/1000.
Reactivity Human, Mouse
Antibody Host Rabbit
Clonality Polyclonal
Specificity This antibody detects endogenous levels of CEP290 protein.
(region surrounding Phe812)
Buffer 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.
Shipping Blue Ice
Predicted Protein Size ~ 290 kDa
Gene Name centrosomal protein 290
Database Link
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
Protein Categories Intracellular Proteins, Nervous system Diseases
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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.

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