Human CEP290 protein (Recombinant) (N-His) (STJP008353)

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STJP008353
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Host: E. coli
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Short Description : Recombinant-Human CEP290-N-His protein was developed from e. coli and has a target region of N-His. For use in research applications.
Formulation: Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Storage Instruction: Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at-20 to-80°C for twelve months from the date of receipt.
Gene Symbol: CEP290
Gene ID: 80184
Uniprot ID: CE290_HUMAN
Immunogen Region: Thr1289-Arg1416
Immunogen: Homo sapiens (Human)
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 Protein Names Centrosomal Protein Of 290 Kda protein
Cep290 protein
Bardet-Biedl Syndrome 14 Protein protein
Cancer/Testis Antigen 87 protein
Ct87 protein
Nephrocystin-6 protein
Tumor Antigen Se2-2 protein
CEP290 protein
BBS14 protein
KIAA0373 protein
NPHP6 protein

Information sourced from Uniprot.org