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Rabbit Anti-CEP290 Polyclonal Antibody#abs140994

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Description

Catalog-specification	Delivery time	USD price
abs140994-100ug	1-2 Weeks	301.0
abs140994-50ug	1-2 Weeks	201.0

Overview
catalog	abs140994
Other names	Possible highly similar content generated by rearranging the provided information: The protein known as Cep290, or centrosomal protein of 290 kDa, is encoded by the KIAA0373 gene and also called Bardet-Biedl syndrome 14 protein (BBS14) due to its involvement in this rare genetic disorder. In addition, Cep290 is part of the nephrocystin family and designated as the sixth member (NPHP6), being associated with a form of inherited blindness called Leber congenital amaurosis type 10 (LCA10) and another ciliopathy called Meckel syndrome type 4 (MKS4). The function of Cep290 revolves around regulating the centrosome, a key organelle involved in cell division and organization of the microtubule cytoskeleton, as well as modulating the primary cilium, a specialized antenna-like structure that senses extracellular signals and relays them intracellularly. The role of Cep290 in the primary cilium is particularly relevant for the development and maintenance of photoreceptor cells in the retina and the renal tubules in the kidney, where cilia play crucial roles in fluid homeostasis and signaling. Interestingly, Cep290 is also expressed in testis and belongs to the cancer/testis antigen 87 (CT87) group, which means that it may have immunogenic properties and elicit immune responses in some cancer patients. Moreover, mutations in Cep290 have been linked to several other ciliopathies, such as Joubert syndrome (JBTS5, JBTS6), Senior-Loken syndrome (SLSN6 1, 2, 5), and possibly some forms of autism spectrum disorders (ASD). Thus, Cep290 is a multifaceted protein that plays diverse roles in various organs and diseases, making it a promising target for therapeutic intervention and diagnostic biomarkers.
Source	Rabbit
Specificity	The CEP290 Antibody has the capacity to detect the total amount of CEP290 that is present in the organism naturally. To generate content that is highly similar to the original information provided, one can shuffle the words around while ensuring that the meaning and message of the text are preserved.
Species Reactivity	Human;Mouse
Predictive reaction species	Sheep;Rabbit;Horse;Pig;
Antigen	CEP290
Application	WB 1:500~1:1000，IF/ICC 1:100-1:500ELISA(peptide) 1:20000-1:40000。，。，。，。
Immunogen	A synthesized peptide.
MW	290 KD
Properties
Concentration	1mg/ml
purification	To purify the antiserum, the SulfoLink™ Coupling Resin was utilized in peptide affinity chromatography method.
Clonality	Polyclonal Antibody
Stability & Storage	It is advised to store at -20 °C for a duration of one year. To maintain the integrity of the substance, it is crucial to avoid subjecting it to multiple freeze/thaw cycles.
Storage buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt.
Target
Background	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).
Tissue specificity	Ubiquitous. Expressed strongly in placenta and weakly in brain.
Posttranslational modification	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 it displacement from centriolar satellites.
Celluar localization	Cytoskeleton;Cytosol;Extracellular region or secreted;Nucleus;
UniPort	O15078