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    cep290-antibody-ab84870.pdf

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Anti-CEP290 antibody (ab84870)

  • Datasheet
  • SDS
Reviews (1) Submit a question References (29)

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Immunocytochemistry/ Immunofluorescence - Anti-CEP290 antibody (ab84870)

    Key features and details

    • Rabbit polyclonal to CEP290
    • Suitable for: ICC/IF
    • Reacts with: Human
    • Isotype: IgG

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    Recombinant Human CEP290 protein (ab164215)

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    Overview

    • Product name

      Anti-CEP290 antibody
      See all CEP290 primary antibodies
    • Description

      Rabbit polyclonal to CEP290
    • Host species

      Rabbit
    • Tested applications

      Suitable for: ICC/IFmore details
    • Species reactivity

      Reacts with: Human
      Predicted to work with: Horse, Rhesus monkey, Gorilla, Orangutan, Elephant
    • Immunogen

      Synthetic peptide corresponding to a region between residues 2429 and 2479 of human CEP290 (NP_079390.3).

    • General notes

      The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

      If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

    Properties

    • Form

      Liquid
    • Storage instructions

      Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
    • Storage buffer

      pH: 6.8
      Preservative: 0.09% Sodium azide
      Constituents: 0.1% BSA, Tris buffered saline
    • Concentration information loading...
    • Purity

      Immunogen affinity purified
    • 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.
    • Clonality

      Polyclonal
    • Isotype

      IgG
    • Research areas

      • Neuroscience
      • Sensory System
      • Visual system
      • Neuroscience
      • Sensory System
      • Olfactory system
      • Epigenetics and Nuclear Signaling
      • Transcription
      • Other factors

    Associated products

    • Compatible Secondaries

      • Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077)
      • Goat Anti-Rabbit IgG H&L (HRP) (ab205718)
    • Isotype control

      • Rabbit IgG, polyclonal - Isotype Control (ChIP Grade) (ab171870)
    • Recombinant Protein

      • Recombinant Human CEP290 protein (ab164215)

    Applications

    The Abpromise guarantee

    Our Abpromise guarantee covers the use of ab84870 in the following tested applications.

    The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

    Application Abreviews Notes
    ICC/IF
    1/100 - 1/500. Fix with formaldehyde. Acetone fixation is not recommended.
    Notes
    ICC/IF
    1/100 - 1/500. Fix with formaldehyde. Acetone fixation is not recommended.

    Target

    • Function

      Activates ATF4-mediated transcription. Required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes.
    • Tissue specificity

      Ubiquitous. Expressed strongly in placenta and weakly in brain.
    • Involvement in disease

      Defects in CEP290 are a cause of Joubert syndrome type 5 (JBTS5) [MIM:610188]. Joubert syndrome is an autosomal recessive disease characterized by cerebellar vermis hypoplasia with prominent superior cerebellar peduncles (the 'molar tooth sign' on axial magnetic resonance imaging), psychomotor delay, hypotonia, ataxia, oculomotor apraxia and neonatal breathing abnormalities. JBTS5 shares the neurologic and neuroradiologic features of Joubert syndrome together with severe retinal dystrophy and/or progressive renal failure characterized by nephronophthisis.
      Defects in CEP290 are a cause of Senior-Loken syndrome type 6 (SLSN6) [MIM:610189]. Senior-Loken syndrome is also known as juvenile nephronophthisis with Leber amaurosis. It is an autosomal recessive renal-retinal disorder, characterized by progressive wasting of the filtering unit of the kidney, with or without medullary cystic renal disease, and progressive eye disease.
      Defects in CEP290 are the cause of Leber congenital amaurosis type 10 (LCA10) [MIM:611755]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children.
      Defects in CEP290 are the cause of Meckel syndrome type 4 (MKS4) [MIM:611134]. MKS4 is an autosomal recessive disorder characterized by a combination of renal cysts and variably associated features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia and cysts, and polydactyly.
      Note=Antibodies against CEP290 are present in sera from patients with cutaneous T-cell lymphomas, but not in the healthy control population.
      Defects in CEP290 are the cause of Bardet-Biedl syndrome type 14 (BBS14) [MIM:209900]. A syndrome characterized by usually severe pigmentary retinopathy, early-onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. Inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for disease manifestation in some cases (triallelic inheritance).
    • Cellular localization

      Cytoplasm > cytoskeleton > centrosome. Nucleus. Cell projection > cilium. Connecting cilium of photoreceptor cells, base of cilium in kidney intramedullary collecting duct cells.
    • Target information above from: UniProt accession O15078 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Database links

      • Entrez Gene: 80184 Human
      • Omim: 610142 Human
      • SwissProt: O15078 Human
      • Unigene: 150444 Human
      • Alternative names

        • 3H11AG antibody
        • Bardet-Biedl syndrome 14 protein antibody
        • BBS14 antibody
        • Cancer/testis antigen 87 antibody
        • CE290_HUMAN antibody
        • Centrosomal protein 290 antibody
        • Centrosomal protein 290kDa antibody
        • Centrosomal protein of 290 kDa antibody
        • Cep290 antibody
        • CT87 antibody
        • CTCL tumor antigen se2 2 antibody
        • FLJ13615 antibody
        • FLJ21979 antibody
        • JBTS5 antibody
        • JBTS6 antibody
        • KIAA0373 antibody
        • LCA10 antibody
        • Meckel syndrome, type 4 antibody
        • MKS4 antibody
        • Monoclonal antibody 3H11 antigen antibody
        • Nephrocystin 6 antibody
        • Nephrocystin-6 antibody
        • NPHP6 antibody
        • POC3 antibody
        • POC3 centriolar protein homolog antibody
        • Prostate cancer antigen T21 antibody
        • rd16 antibody
        • SLSN6 1, 2, 5 antibody
        • SLSN6 antibody
        • Tumor antigen se2-2 antibody
        see all

      Images

      • Immunocytochemistry/ Immunofluorescence - Anti-CEP290 antibody (ab84870)
        Immunocytochemistry/ Immunofluorescence - Anti-CEP290 antibody (ab84870)
        Staining of human CEP290 in NBF fixed asynchronous HeLa cells by Immunocytochemistry, using ab84870 at a dilution of 1/250. Detection: Red fluorescent goat anti-rabbit IgG used at a dilution of 1/100.

      Protocols

      • Immunocytochemistry & immunofluorescence protocols

      Click here to view the general protocols

      Datasheets and documents

      • SDS download

      • Datasheet download

        Download

      References (29)

      Publishing research using ab84870? Please let us know so that we can cite the reference in this datasheet.

      ab84870 has been referenced in 29 publications.

      • Chen C  et al. Ciliopathy protein HYLS1 coordinates the biogenesis and signaling of primary cilia by activating the ciliary lipid kinase PIPKI?. Sci Adv 7:N/A (2021). PubMed: 34162535
      • Wiegering A  et al. Rpgrip1l controls ciliary gating by ensuring the proper amount of Cep290 at the vertebrate transition zone. Mol Biol Cell 32:675-689 (2021). PubMed: 33625872
      • Yan H  et al. TALPID3 and ANKRD26 selectively orchestrate FBF1 localization and cilia gating. Nat Commun 11:2196 (2020). PubMed: 32366837
      • Aydin ÖZ  et al. Acute inhibition of centriolar satellite function and positioning reveals their functions at the primary cilium. PLoS Biol 18:e3000679 (2020). PubMed: 32555591
      • Sullenberger C  et al. With Age Comes Maturity: Biochemical and Structural Transformation of a Human Centriole in the Making. Cells 9:N/A (2020). PubMed: 32526902
      View all Publications for this product

      Customer reviews and Q&As

      Show All Reviews Q&A
      Submit a review Submit a question

      Western blot abreview for Anti-CEP290 antibody

      Excellent
      Abreviews
      Abreviews
      abreview image
      Application
      Western blot
      Sample
      Human Cell lysate - whole cell (hek293 CELLS)
      Gel Running Conditions
      Reduced Denaturing (10%)
      Loading amount
      40 µg
      Specification
      hek293 CELLS
      Blocking step
      Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 20% · Temperature: RT°C
      Read More
      The reviewer received a reward from Abcam’s Loyalty Program in thanks for submitting this Abreview and for helping the scientific community make better-informed decisions.

      Dr. Amani Hassan

      Verified customer

      Submitted Mar 25 2017

      Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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