CEP290 Antibody (G-4): sc-390637
- CEP290 Antibody (G-4) is a mouse monoclonal IgG2a κ, cited in 1 publications, provided at 200 µg/ml
- raised against amino acids 1441-1740 mapping within an internal region of CEP290 of human origin
- recommended for detection of CEP290 of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA
- m-IgG Fc BP-HRP and m-IgG2a BP-HRP are the preferred secondary detection reagents for CEP290 Antibody (G-4) for WB and IHC(P) applications. These reagents are now offered in bundles with CEP290 Antibody (G-4) (see ordering information below).
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CEP290 Antibody (G-4) is a mouse monoclonal IgG2a antibody that detects CEP290 in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). CEP290, also known as centrosomal protein of 290 kDa, plays a crucial role in the proper functioning of cilia, which are essential for various cellular processes, including signaling pathways and the maintenance of cellular homeostasis. CEP290 is primarily located at the centrosome and is vital for the assembly and stability of ciliary structures, ensuring the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells. Disruptions in CEP290 function due to mutations can lead to severe developmental disorders, such as Joubert syndrome and Leber congenital amaurosis, highlighting CEP290′s importance in both cellular architecture and human health. Anti-CEP290 antibody (G-4) enables researchers to gain insights into CEP290′s role in these critical processes and its implications in disease mechanisms.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
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CEP290 Antibody (G-4) References:
- CEP290 gene transfer rescues Leber congenital amaurosis cellular phenotype. | Burnight, ER., et al. 2014. Gene Ther. 21: 662-72. PMID: 24807808
- CEP290 alleles in mice disrupt tissue-specific cilia biogenesis and recapitulate features of syndromic ciliopathies. | Rachel, RA., et al. 2015. Hum Mol Genet. 24: 3775-91. PMID: 25859007
- Joubert syndrome: a model for untangling recessive disorders with extreme genetic heterogeneity. | Bachmann-Gagescu, R., et al. 2015. J Med Genet. 52: 514-22. PMID: 26092869
- LEBER CONGENITAL AMAUROSIS DUE TO CEP290 MUTATIONS-SEVERE VISION IMPAIRMENT WITH A HIGH UNMET MEDICAL NEED: A Review. | Leroy, BP., et al. 2021. Retina. 41: 898-907. PMID: 33595255
- A novel CEP290 disease-causing variant identified in a patient with leber congenital amaurosis using a medical diagnostic panel sequencing. | Chen, BB., et al. 2022. Ophthalmic Genet. 43: 97-103. PMID: 34809537
- Fleck-like lesions in CEP290-associated leber congenital amaurosis: a case series. | Aleman, TS., et al. 2022. Ophthalmic Genet. 43: 824-833. PMID: 36469661
- Eupatilin improves cilia defects in human CEP290 ciliopathy models. | Corral-Serrano, JC., et al. 2023. bioRxiv.. PMID: 37205323
- Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models. | Corral-Serrano, JC., et al. 2023. Cells. 12: PMID: 37371046
- Cep131-Cep162 and Cby-Fam92 complexes cooperatively maintain Cep290 at the basal body and contribute to ciliogenesis initiation. | Wu, Z., et al. 2024. PLoS Biol. 22: e3002330. PMID: 38442096
- Spectrum and frequencies of extraocular features reported in CEP290-associated ciliopathy - A systematic review. | Vrabič, N., et al. 2024. J Fr Ophtalmol. 47: 104232. PMID: 39213781
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
CEP290 Antibody (G-4) | sc-390637 | 200 µg/ml | $322.00 | |||
CEP290 Antibody (G-4): m-IgG Fc BP-HRP Bundle | sc-540826 | 200 µg Ab; 10 µg BP | $361.00 | |||
CEP290 Antibody (G-4): m-IgG2a BP-HRP Bundle | sc-546963 | 200 µg Ab; 10 µg BP | $361.00 |