|Positive WB detected in||HeLa cells|
|Positive IHC detected in||human placenta tissue|
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
|Positive IF detected in||HeLa cells|
|Western Blot (WB)||WB : 1:500-1:1000|
|Immunohistochemistry (IHC)||IHC : 1:20-1:200|
|Immunofluorescence (IF)||IF : 1:50-1:500|
|Sample-dependent, check data in validation data gallery|
The immunogen of 22490-1-AP is CEP290 Fusion Protein expressed in E. coli.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||human, mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||CEP290 fusion protein Ag17457|
|Full Name||centrosomal protein 290kDa|
|Calculated molecular weight||2479 aa, 290 kDa|
|Observed molecular weight||290 kDa, 150-180 kDa, 80 kDa, 60 kDa|
|GenBank accession number||BC008641|
|Gene ID (NCBI)||80184|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
Studies in the Chlamydomonas model system has shown that the ciliary protein CEP290 is a critical component of these Y-link junctions. Additionally, numerous studies have demonstrated that CEP290’s function is critical for IFT—in CEP290 knockdown experiments, many proteins that would normally localize to the cilium fail to do so, and cilium formation is disrupted or absent. Mutations in CEP290 are accountable for cases of nephronophthisis, Leber congenital amaurosis and Joubert syndrome. In IF analtsis of HeLa cells, the white arrows show centrosome and cilium staining. Two isoforms were produced by alternative splicing with predicted MW of 290 and 180 kDa according to UniProt. Catalog#22490-1-AP can recognise both.
Centriolar satellites are required for efficient ciliogenesis and ciliary content regulation.
Centrosomal and ciliary targeting of CCDC66 requires cooperative action of centriolar satellites, microtubules and molecular motors.
J Biol Chem
The myosin-tail homology domain of centrosomal protein 290 is essential for protein confinement between the inner and outer segments in photoreceptors.
J Biol Chem
DAZ interacting protein 1 (Dzip1) phosphorylation by Polo-like kinase 1 (Plk1) regulates the centriolar satellites localization of the BBSome during the cell cycle.
Trichoplein binds PCM1 and controls endothelial cell function by regulating autophagy.
A CEP290 C-Terminal Domain Complements the Mutant CEP290 of Rd16 Mice In Trans and Rescues Retinal Degeneration.
The reviews below have been submitted by verified Proteintech customers who received an incentive forproviding their feedback.
Jay (Verified Customer) (11-08-2019)
Human RPE1 cell line with Methanol fixation. Immunostaining result shows that CEP290 localizes to centrosome (Red; CEP290, Green; CEP164 for Centrosome marker, Blue;DAPI for Nucleus). The antibody seems to detect additional nucleoli signals.