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CEP290 Antibody

Images

 
Western Blot: CEP290 Antibody [NB100-86991] - Representative examples of immunoblot images of purified olfactory cilia. Three distinct cilia preparations each from LRBA-KO and WT mice (age, 4 weeks) were blotted in ...read more
Immunocytochemistry/ Immunofluorescence: CEP290 Antibody [NB100-86991] - NBF-fixed asynchronous HeLa cells. Antibody used at a dilution of 1:500.
Western Blot: CEP290 Antibody [NB100-86991] - Species: Mouse, Cell line: Brain homogenate, Primary antibody dilution: 1:400. Band detected at 300KDa. This image has been provided by Manfred W. Kilimann, MD, PhD, ...read more
Western Blot: CEP290 Antibody [NB100-86991] - Detection of human CEP290 by western blot and immunoprecipitation. Samples: Whole cell lysate from HeLa (5, 15 and 50 ug for WB; 1 mg for IP, 20% of IP loaded) and HEK293T ...read more

Product Details

Summary
Reactivity Hu, MuSpecies Glossary
Applications WB, ICC/IF, IP
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated
Concentration
1.0 mg/ml

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CEP290 Antibody Summary

Immunogen
The immunogen recognized by this antibody maps to a region between residue 2429 and 2479 of human centrosomal protein 290kDa using the numbering given in entry NP_079390.3 (GeneID 80184).
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
CEP290
Purity
Immunogen affinity purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Immunocytochemistry/ Immunofluorescence 1:250-1:1000
  • Immunoprecipitation 2-5 ug/mg lysate
  • Western Blot 1:2000-1:10000
Application Notes
ICC/IF (PMID: 21524666). CEP290 antibody validated for WB from a verified customer review. Formaldehyde fixation is recommended. Permeabilization with Triton-X 100 is recommended for formaldehyde-fixed cells.
Theoretical MW
290 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
Publications
Read Publications using
NB100-86991 in the following applications:

  • 2 publications
  • WB
    6 publications

Reactivity Notes

Mouse reactivity reported from a verified customer review.

Packaging, Storage & Formulations

Storage
Store at 4C. Do not freeze.
Buffer
Tris-Citrate/Phosphate (pH 7.0 - 8.0)
Preservative
0.09% Sodium Azide
Concentration
1.0 mg/ml
Purity
Immunogen affinity purified

Alternate Names for CEP290 Antibody

  • BBS14Bardet-Biedl syndrome 14 protein
  • Cancer/testis antigen 87
  • centrosomal protein 290kDa
  • Cep290
  • CT87JBTS6
  • FLJ13615
  • JBTS5CTCL tumor antigen se2-2
  • KIAA0373FLJ21979
  • LCA10monoclonal 3H11 antigen
  • MKS4
  • Nephrocystin-6
  • NPHP6centrosomal protein of 290 kDa
  • POC3 centriolar protein homolog
  • POC3
  • prostate cancer antigen T21
  • rd16,3H11AG
  • SLSN6nephrocytsin-6,3H11Ag
  • Tumor antigen se2-2

Background

This 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. Mutations in this gene have been associated with Joubert syndrome and nephronophthisis and the presence of antibodies against this protein is associated with several forms of cancer. [provided by RefSeq]

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Applications: WB, ICC/IF, IP

Publications for CEP290 Antibody (NB100-86991)(12)

We have publications tested in 2 confirmed species: Human, Mouse.

We have publications tested in 2 applications: ICC/IF, WB.


Filter By Application
ICC/IF
(2)
WB
(6)
All Applications
Filter By Species
Human
(6)
Mouse
(5)
All Species
Showing Publications 1 - 10 of 12. Show All 12 Publications.
Publications using NB100-86991 Applications Species
Speight P, Rozycki M, Venugopal S et al. Myocardin-related transcription factor and serum response factor regulate cilium turnover by both transcriptional and local mechanisms iScience 2021-07-01 [PMID: 34278253] (WB, Human, Mouse) WB Human, Mouse
Slaats GG, Saldivar JC, Bacal J et al. DNA replication stress underlies renal phenotypes in CEP290-associated Joubert syndrome. J. Clin. Invest. 2015-08-24 [PMID: 26301811] (WB, Human) WB Human
Alby C, Piquand K, Huber C et al. Mutations in KIAA0586 Cause Lethal Ciliopathies Ranging from a Hydrolethalus Phenotype to Short-Rib Polydactyly Syndrome. Am J Hum Genet 2015-08-06 [PMID: 26166481] (Human) Human
Hopp K, Heyer C, Hommerding C et al. B9D1 is revealed as a novel Meckel syndrome (MKS) gene by targeted exon-enriched next-generation sequencing and deletion analysis Hum Mol Genet 2011-07-01 [PMID: 21493627] (ICC/IF, Human) ICC/IF Human
Matsuo K, Nakajima Y, Shigeta M et al. Ciliary protein CEP290 regulates focal adhesion via microtubule system in non-ciliated cells1 bioRxiv 2023-04-03
Julio C. Corral-Serrano, Ideke J. C. Lamers, Jeroen van Reeuwijk, Lonneke Duijkers, Anita D. M. Hoogendoorn, Adem Yildirim, Nikoleta Argyrou, Renate A. A. Ruigrok, Stef J. F. Letteboer, Rossano Butcher, Max D. van Essen, Sanae Sakami, Sylvia E. C. van Beersum, Krzysztof Palczewski, Michael E. Cheetham, Qin Liu, Karsten Boldt, Uwe Wolfrum, Marius Ueffing, Alejandro Garanto, Ronald Roepman, Rob W. J. Collin PCARE and WASF3 regulate ciliary F-actin assembly that is required for the initiation of photoreceptor outer segment disk formation Proceedings of the National Academy of Sciences of the United States of America 2020-05-05 [PMID: 32312818]
Dulla K, Aguila M, Lane A et al. Splice-Modulating Oligonucleotide QR-110 Restores CEP290 mRNA and Function in Human c.2991 and 1655 A to G LCA10 Models Mol Ther Nucleic Acids 2018-07-23 [PMID: 30114557] (WB, Mouse) WB Mouse
Barny I, Perrault I, Michel C et al. Basal exon skipping and nonsense-associated altered splicing allows bypassing complete CEP290 loss-of-function in individuals with unusually mild retinal disease. Hum Mol Genet 2018-05-16 [PMID: 29771326] (Human) Human
Kurtenbach S, Giebl A, Stromberg S et al. The BEACH Protein LRBA Promotes the Localization of the Heterotrimeric G-protein Golf to Olfactory Cilia Sci Rep 2017-08-16 [PMID: 28814779] (WB, Mouse) WB Mouse
.Bangs FK, Schrode N, Hadjantonakis AK, Anderson KV. Lineage specificity of primary cilia in the mouse embryo. Nat Cell Biol 2015-02-01 [PMID: 25599390] (ICC/IF, Mouse) ICC/IF Mouse
Show All 12 Publications.

Reviews for CEP290 Antibody (NB100-86991) (0)

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Product General Protocols

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Video Protocols

WB Video Protocol
ICC/IF Video Protocol

FAQs for CEP290 Antibody (NB100-86991). (Showing 1 - 1 of 1 FAQs).

  1. Can we do generation of blocking peptide on request for NB100-86991 ?
    • Unfortunately, we do not offer the blocking peptide. 

Secondary Antibodies

 

Isotype Controls

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Research Areas for CEP290 Antibody (NB100-86991)

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Blogs on CEP290.

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Bioinformatics

Gene Symbol CEP290
Entrez
Uniprot