Trial size CEP290 antibodies available in the Antibody Validation Project

Introduction:

The CEP290 gene encodes the Centrosomal protein of 290 kDa, responsible for early and late cilia formation, cilia inhibition, ciliogenesis and the disappearance of centriolar satellites. It can be found mainly in cell centrosomes and mutations of CEP290 are related to several syndromes, including Joubert syndrome, Bardet-Biedl Syndrome 14, Leber Congenital Amaurosis 10 and Meckel Syndrome, Type 4. Furthermore, presence of antibodies for this gene in the human organism is associated with several cancers and we provide a number of trial sized antibodies, which are suitable for CEP290 research purposes, and to help you make the right decision and find the best suited antibody for you, please find the answers to some of the key topics regarding CEP290 that we have provided in the links below.

• What is CEP290?
• Where is CEP290 found?
• What role does CEP290 play in disease?
• CEP290 antibodies in the Antibody Validation project
• Customer reviewed CEP290 antibodies
• Other CEP290 antibodies available in the Antibody Validation Project
• Popular CEP290 antibodies not currently in the Antibody Validation Project
• How does the Antibody Validation Project work?

What is CEP290?

The CEP290 gene encodes a protein with 13 putative coiled-coil domains, a region with homology to SMC chromosome segregation ATPases, six KID motifs, three tropomyosin homology domains and an ATP/GTP binding site motif A. The protein is localized to the centrosome and cilia and has sites for N-glycosylation, tyrosine sulfation, phosphorylation, N-myristoylation, and amidation. The CEP290 protein is also involved in early and late steps in cilia formation, and its association with CCP110 is required for inhibition of primary cilia formation by CCP110. Also, it 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). Furthermore, it is required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins to centrosomes such as of PCM. This protein plays an important role for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells, and it may also play a role in ciliary transport processes. 

Where is CEP290 found?

Being a centrosomal protein, CEP290 is mainly found in the centrosome of the cells. On tissue level, it has cytoplasmic expression in most tissues with nuclear expression in some cell types.

What role does CEP290 play in disease?

 Mutations in this gene have been associated with Joubert syndrome, presenting with cerebellar ataxia, neonatal breathing abnormalities and hypotonia, and the presence of antibodies against this protein is associated with several forms of cancer. Furthermore, Senior-Loken syndrome 6 is closely related to CEP290, because this gene and the protein synthesised are one of the major reasons for this renal-retinal disorder characterised by progressive wasting of the nephronophthisis and progressive eye disease, usually present during the first year after birth. Apart from that, Bardet-Biedl Syndrome 14, Leber Congenital Amaurosis 10 and Meckel Syndrome, Type 4 are also related to CEP290.

CEP290 antibodies in the Antibody Validation project

St John's Laboratory offers a variety of trial size primary antibodies which target CEP290, which are available to try through the Antibody Validation Project. Below we highlight those which have already been reviewed, as well as others which are available to try.

Customer reviewed CEP290 antibodies

Model	Name	Host	Clonality	Rating	Application Tested
STJ92224	Anti-CEP290 antibody	Rabbit	Polyclonal	4	IF

Other CEP290 antibodies available in the Antibody Validation Project

Model	Name	Host	Clonality	Recommended Applications
STJ92224	Anti-CEP290 antibody	Rabbit	Polyclonal	WB,ELISA,IF

Popular CEP290 antibodies not currently in the Antibody Validation Project

Model	Name	Host	Clonality	Recommended Applications
STJ118587	Anti-CEP290 Antibody	Rabbit	Polyclonal	WB
STJ71490	Anti-CEP290 Antibody	Goat	Polyclonal	ELISA
STJ71812	Anti-CEP290 Antibody	Goat	Polyclonal	ELISA,FC

How does the Antibody Validation Project work?

In order to help our customers with their choice of antibody with low risk, we offer one of the largest ranges of primary antibodies with trial size options available.

The Antibody Validation Project works like this.
1. Order your trial size antibody online.
2. Explore its suitability.
3. Return your results by posting them on the product page.
4. Receive a sample refund.

Find out more here.