Abcam, ab84870, Anti-CEP290 antibody

Size: 100µL
Rabbit Polyclonal CEP290 antibody. Suitable for ICC/IF, WB and reacts with Human samples. Cited in 39 publications. Immunogen corresponding to Synthetic Peptide within Human CEP290 aa 2400 to C-terminus.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:ICC/IF, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Synthetic Peptide within Human CEP290 aa 2400 to C-terminus. The exact immunogen used to generate this antibody is proprietary information.O15078

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Purification notes-Purified using an epitope specific to CEP290 immobilized on solid support. Antibody concentration was determined by extinction coefficient: absorbance at 280 nm of 1.4 equals 1.0 mg of IgG., Storage buffer-pH: 6.8 - 7.4Preservative: 0.09% Sodium azideConstituents: Tris buffered saline, 0.1% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
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.
Biological function summary
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.
Pathways
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.