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

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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NB100-86991
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Applications: WB, ICC/IF, IP

Publications for CEP290 Antibody (NB100-86991)(10)

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 10.
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 Jul 1 2021 [PMID: 34278253] (WB, Mouse, Human) WB Mouse, Human
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 Jul 23 2018 [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. May 16 2018 [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 Aug 16 [PMID: 28814779] (WB, Mouse) WB Mouse
Slaats GG, Saldivar JC, Bacal J et al. DNA replication stress underlies renal phenotypes in CEP290-associated Joubert syndrome. J. Clin. Invest. 2015 Aug 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 Aug 6 [PMID: 26166481] (Human) Human
Bangs FK, Schrode N, Hadjantonakis AK, Anderson KV. Lineage specificity of primary cilia in the mouse embryo Nat. Cell Biol. 2015 Feb 01 [PMID: 25599390] (ICC/IF, Mouse) ICC/IF Mouse
Garanto A, van Beersum SE, Peters TA et al. Unexpected CEP290 mRNA Splicing in a Humanized Knock-In Mouse Model for Leber Congenital Amaurosis. PLoS One. 2013 Nov 6 [PMID: 24223178] (WB, Mouse) WB Mouse
AON-mediated Exon Skipping Restores Ciliation in Fibroblasts Harboring the Common Leber Congenital Amaurosis CEP290 Mutation Gerard, X, Perrault Mol Ther Nucleic Acids [PMID: 23344081] (WB, Human) WB 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 Jul 1 [PMID: 21493627] (ICC/IF, Human) ICC/IF Human

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

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Product General Protocols

Find general support by application which include: protocols, troubleshooting, illustrated assays, videos and webinars.

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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Blogs on CEP290.

LAMP2: Protector of the lysosome
LAMP2 belongs to the family of membrane glycoproteins who confer selectins with carbohydrate ligands. LAMP2 has been implicated in tumor cell metastasis, as well as overall protection, maintenance, and adhesion of the lysosome. It appears that LAMP2 m...  Read full blog post.

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Bioinformatics

Gene Symbol CEP290
Entrez
Uniprot