Rabbit Polyclonal CEP290 antibody. Suitable for WB, IHC-P and reacts with Human samples. Immunogen corresponding to Synthetic Peptide within Human CEP290 aa 750-850.
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol (glycerin, glycerine), 49% PBS, 0.87% Sodium chloride
WB | IHC-P | |
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Human | Tested | Tested |
Mouse | Predicted | Predicted |
Species | Dilution info | Notes |
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Species Human | Dilution info 1/500.00000 - 1/1000.00000 | Notes - |
Species | Dilution info | Notes |
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Species Mouse | Dilution info - | Notes - |
Species | Dilution info | Notes |
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Species Human | Dilution info 10 µg/mL | Notes - |
Species | Dilution info | Notes |
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Species Mouse | Dilution info - | Notes - |
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Involved in early and late steps in cilia formation. Its association with CCP110 is required for inhibition of primary cilia formation by CCP110 (PubMed:18694559). 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 (PubMed:24421332). Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes (By similarity). Required for efficient recruitment of RAB8A to primary cilium (PubMed:17705300). 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 (By similarity). 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 (PubMed:25552655). Activates ATF4-mediated transcription (PubMed:16682973).
BBS14, KIAA0373, NPHP6, CEP290, Centrosomal protein of 290 kDa, Cep290, Bardet-Biedl syndrome 14 protein, Cancer/testis antigen 87, Nephrocystin-6, Tumor antigen se2-2, CT87
Rabbit Polyclonal CEP290 antibody. Suitable for WB, IHC-P and reacts with Human samples. Immunogen corresponding to Synthetic Peptide within Human CEP290 aa 750-850.
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol (glycerin, glycerine), 49% PBS, 0.87% Sodium chloride
CEP290 also known as centrosomal protein of 290 kDa acts as an important structural component in cells. It has an approximate mass of 290 kilodaltons and is primarily expressed in ciliated cells across various tissues including the retina and kidneys. CEP290 localizes at the centrosome and is integral to the formation and stability of cilia and flagella. The protein plays an important mechanic role in maintaining the structural integrity of cilia which are essential for cellular signaling and motility.
CEP290 contributes significantly to the formation and maintenance of the primary cilium a cellular organelle. It forms part of the transition zone complex a critical region at the base of cilia. This zone functions as a barrier regulating the movement of molecules in and out of the cilia. As a result CEP290 supports the transport and localization of ciliary proteins affecting ciliary homeostasis and signal transduction mechanisms within the cell.
CEP290 is intricately involved in the ciliary transport and the phototransduction pathway within photoreceptor cells. CEP290 operates in coordination with proteins such as RPGR in these pathways ensuring proper ciliary trafficking and function. This activity is important for sensory perception processes including vision. The disruption of CEP290 affects these pathways leading to compromised sensory functions.
CEP290 mutations associate with specific ciliopathies such as Joubert syndrome and Leber congenital amaurosis. These conditions result from defective ciliary function and altered cellular pathways. In the context of Joubert syndrome CEP290 interacts with proteins such as AHI1 where both play roles in neurodevelopment and function. The understanding of these interactions provides insights into the mechanistic implications of CEP290-related disorders.
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All lanes: Western blot - Anti-CEP290 antibody (ab190237) at 1/500 dilution
Lane 1: K562 cell extract
Lane 2: K562 cell extract with synthesized peptide
Predicted band size: 290 kDa
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human pancreas tissue labeling CEP290 with ab190237 at 10μg/ml.
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