Anti-CEP290 antibody (740-820 Internal)

Clonality:	Polyclonal
Conjugation:	Unconjugated
Isotype:	IgG
Formulation:	Liquid in PBS containing 50% Glycerol, 0.5% BSA and 0.02% Sodium Azide.
Purification:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:	1 mg/mL
Dilution Range:	WB 1:500-1:2000 ELISA 1:20000
Storage Instruction:	Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles.

Gene Symbol:	CEP290
Gene ID:	80184
Uniprot ID:	CE290_HUMAN
Immunogen Region:	771-820 aa
Specificity:	CEP290 Polyclonal Antibody detects endogenous levels of CEP290 protein.
Immunogen:	The antiserum was produced against synthesized peptide derived from the human CEP290 at the amino acid range 771-820

Post Translational Modifications	Ubiquitinated. May undergo monoubiquitination.monoubiquitination is inhibited in response to cellular stress, such as ultraviolet light (UV) radiation or heat shock, but does not cause its displacement from centriolar satellites.
Function	Involved in early and late steps in cilia formation. Its association with CCP110 is required for inhibition of primary cilia formation by CCP110. May play a role in early ciliogenesis in the disappearance of centriolar satellites and in the transition of primary ciliar vesicles (PCVs) to capped ciliary vesicles (CCVs). Required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins to centrosomes such as of PCM1. Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells.may play a role in ciliary transport processes. Required for efficient recruitment of RAB8A to primary cilium. In the ciliary transition zone is part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition. Involved in regulation of the BBSome complex integrity, specifically for presence of BBS2, BBS5 and BBS8/TTC8 in the complex, and in ciliary targeting of selected BBSome cargos. May play a role in controlling entry of the BBSome complex to cilia possibly implicating IQCB1/NPHP5. Activates ATF4-mediated transcription.
Protein Name	Centrosomal Protein Of 290 Kda Cep290 Bardet-Biedl Syndrome 14 Protein Cancer/Testis Antigen 87 Ct87 Nephrocystin-6 Tumor Antigen Se2-2
Database Links	Reactome: R-HSA-2565942 Reactome: R-HSA-380259 Reactome: R-HSA-380270 Reactome: R-HSA-380284 Reactome: R-HSA-380320 Reactome: R-HSA-5620912 Reactome: R-HSA-6798695 Reactome: R-HSA-8854518
Cellular Localisation	Cytoplasm Cytoskeleton Microtubule Organizing Center Centrosome Centriolar Satellite Nucleus Cell Projection Cilium Cilium Basal Body Centriole Cytoplasmic Vesicle Displaced From Centriolar Satellites In Response To Cellular Stress Such As Ultraviolet Light (Uv) Radiation Or Heat Shock Found In The 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 Localization At The Ciliary Transition Zone As Well As At Centriolar Satellites Is Bbsome-Dependent
Alternative Antibody Names	Anti-Centrosomal Protein Of 290 Kda antibody Anti-Cep290 antibody Anti-Bardet-Biedl Syndrome 14 Protein antibody Anti-Cancer/Testis Antigen 87 antibody Anti-Ct87 antibody Anti-Nephrocystin-6 antibody Anti-Tumor Antigen Se2-2 antibody Anti-CEP290 antibody Anti-BBS14 antibody Anti-KIAA0373 antibody Anti-NPHP6 antibody

Information sourced from Uniprot.org

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