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ABN1710 Anti-CEP290

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ABN1710
100 µL  
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      Übersicht

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, R, MIF, WB, IHC, IP, ICCRbPurifiedPolyclonal Antibody
      Description
      Catalogue NumberABN1710
      DescriptionAnti-CEP290
      Alternate Names
      • Cep290
      • Bardet-Biedl syndrome 14 protein homolog
      • Nephrocystin-6
      Background InformationCentrosomal protein of 290 kDa (UniProt: Q6A078; also known as Cep290; Bardet-Biedl syndrome 14 protein homolog; Nephrocystin-6) is encoded by the Cep290 (also known as Kiaa0373, Nphp6) gene (Gene ID: 216274) in murine species. Cep290 is a centrosomal protein that is expressed in multiple organs during early postnatal development, with highest levels in the hindbrain region. Cep290 has 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. Cep290 is part of the tectonic-like complex, which is required for tissue-specific ciliogenesis and may also regulate ciliary membrane composition. It is also required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells. Defects in Cep290 gene are known to cause Leber congenital amaurosis (LCA), an early-onset retinal degenerative disease. Mutations in Cep290 gene are also known to cause Joubert syndrome that is characterized by psychomotor delay, hypotonia, ataxia, oculomotor apraxia and neonatal breathing abnormalities.
      References
      Product Information
      FormatPurified
      PresentationPurified rabbit polyclonal antibody in PBS without preservatives with 50% glycerol.
      Quality LevelMQ100
      Applications
      ApplicationAnti-CEP290, Cat. No. ABN1710, is a highly specific rabbit polyclonal antibody that targets Centrosomal protein of 290 kDa and has been tested in and Immunocytochemistry, Immunofluorescence, Immunohistochemistry, Immunoprecipitation, and Western Blotting,
      Key Applications
      • Immunofluorescence
      • Western Blotting
      • Immunohistochemistry
      • Immunoprecipitation
      • Immunocytochemistry
      Application NotesWestern Blotting Analysis: A representative lot detected CEP290 in transfected HEK293 cells (Rachel, R.A., et. al. (2012). J Clin Invest. 122(4):1233-45) and COS-1 cells (Chang, B., et. al. (2006). Hum Mol Genet. 15(11):1847-57).

      Immunoprecipitation Analysis: A representative lot detected CEP290 in WT and rd16 retinal extract(Chang, B., et. al. (2006). Hum Mol Genet. 15(11):1847-57).

      Immunohistochemistry Analysis: A representative lot detected CEP290 in WT mouse retina (Chang, B., et. al. (2006). Hum Mol Genet. 15(11):1847-57).

      Western Blotting Analysis: 1:1,000-1:2,000 dilution from a representative lot detected CEP290 in mouse retina (Courtesy of Dr. Tiansen at NIH).

      Immunofluorescence Analysis: A representative lot detected CEP290 in ciliated sensory cells (Rachel, R.A., et. al. (2012). J Clin Invest. 122(4):1233-45) and WT mouse retina (Chang, B., et. al. (2006). Hum Mol Genet. 15(11):1847-57).

      Immunofluorescence Analysis: 1:500-1:2,000 dilution from a representative lot detected CEP290 in frozen mouse retina (Courtesy of Dr. Tiansen at NIH).

      Immunocytochemistry Analysis: A representative lot detected CEP290 in IMCD-3 cells (Chang, B., et. al. (2006). Hum Mol Genet. 15(11):1847-57).
      Biological Information
      ImmunogenHis-tagged recombinant fragment corresponding to 80 amino acids from the C-terminal half of mouse Centrosomal protein of 290 kDa.
      Epitopeunknown
      ConcentrationPlease refer to lot specific datasheet.
      HostRabbit
      SpecificityThis polyclonal antibody detects centrosomal protein of 290 kDa in HEK293 cells.
      Species Reactivity
      • Human
      • Rat
      • Mouse
      Species Reactivity NoteHuman, Mouse, Rat.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • Cep290
      • Kiaa0373
      • Nphp6
      Purification MethodAffinity Purified
      UniProt Number
      Molecular Weight~260 kDa observed; 289.08 kDa calculated. Uncharacterized bands may be observed in some lysate(s).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in HEK293 cell lysate.

      Western Blotting Analysis: 1:500 dilution of this antibody detected CEP290 in 10 µg of HEK293 cell lysate.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at -20°C from date of receipt.

      Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Bestellnummer GTIN
      ABN1710 04054839153310