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MABN889 Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9

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MABN889
50 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HIHC, WBMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABN889
      DescriptionAnti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9
      Alternate Names
      • C9ORF72/C9RANT (poly-GA)
      • Protein C9orf72
      Background InformationExpansion of a GGGGCC hexanucleotide repeat sequence in the non-coding region of human chromosome 9 open reading frame 72 or C9orf72 (also known as ALSFTD, FTDALS; Gene ID 203228) is the most common genetic abnormality in familial and sporadic frontotemporal dementia (FTD) and motor neuron disease (MND), with amyotrophic lateral sclerosis (ALS) as the most frequent form. The number of hexanucleotide repeats in the normal population ranges from 2 to 24, whereas up to several thousand repeats (700 - 4,400 repeats) are found in the pathologically expanded allele. Unconventional repeat-associated non-ATG-initiated translation (RANT) of the GGGGCC repeats in the three alternate reading frames generates three polypeptides, each composed of repeating units of two amino acids (dipeptide repeats, DPRs), glycine-alanine (GA), glycine-proline (GP), and glycine-arginine (GR), respectively. These DPR proteins form inclusions particularly in the cerebellar cortex, hippocampus and cerebral neocortex. These inclusions are also immunoreactive for markers of the ubiquitin proteasome system (UPS), including ubiquitin, ubiquilins, and p62, but is distinct from inclusions containing TDP-43 that is also found in FTLD-TDP (frontotemporal lobar degeneration with TDP-43 pathology) and ALS cases. Antibodies against poly-GA, poly-GP, and poly-GR sequence are useful for characterizing the neuroanatomical distribution and clinico-pathological association of DPR pathology with C9ORF72 mutations.
      References
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationDetect C9ORF72/C9RANT using this Anti-C9ORF72/C9RANT, clone 5E9 Antibody validated for use in Western Blotting and Immunohistochemistry.
      Key Applications
      • Immunohistochemistry
      • Western Blotting
      Application NotesWestern Blotting Analysis: A representative lot detected recombinant GST fusion with 15 GA repeats (Mackenzie, I.R., et al. (2013). Acta Neuropahol. 126(6):859-879).

      Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT with 15 GA repeats in cerebellum from a C9ORF72 mutation case (Mackenzie, I.R., et al. (2013). Acta Neuropahol. 126(6):859-879).
      Biological Information
      ImmunogenPolyethylene glycol-conjugated linear peptide containing poly-GA sequence.
      EpitopePoly-GA
      Clone5E9
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityThis antibody recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Species Reactivity NoteHuman. Predicted to react with all species
      Antibody TypeMonoclonal Antibody
      Gene Symbol
      • C9orf72
      Purification MethodProtein G Purified
      UniProt Number
      Molecular WeightVariable. Additional uncharacterized bands maybe observed in some lysates.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western blotting using GST fusion recombinant protein with 15 GA repeats.

      Western Blotting Analysis: 0.05 µg/mL of this antibody detected 10 ug of GST fusion recombinant protein with 15 GA repeats
      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 2-8°C from date of receipt.
      Packaging Information
      Material Size50 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      MABN889 04055977171426

      Documentation

      Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 SDS

      Title

      Safety Data Sheet (SDS) 

      Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Certificates of Analysis

      TitleLot Number
      Anti-C9ORF72/C9RANT (poly-GA), -2653164 2653164
      Anti-C9ORF72/C9RANT (poly-GA), -2794074 2794074
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3150354 3150354
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3226534 3226534
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3328868 3328868
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3470729 3470729
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3557714 3557714
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3694727 3694727
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3738412 3738412
      Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3808623 3808623

      Technical Info

      Title
      Characterization of Estrogen Receptor α Phosphorylation Sites in Breast Cancer Tissue Using the SNAP i.d® 2.0 System
      White Paper: Further considerations of antibody validation and usage.

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies