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MABN889-AF488 Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate

Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate, Cat. No. MABN889-AF488, is a highly specific mouse monoclonal antibody that targets Gly-Ala repeats (C9orf72) and has been tested

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MABN889-AF488
100 µL  
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      Overview

      Description
      Catalogue NumberMABN889-AF488
      DescriptionAnti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate
      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.
      Product Information
      FormatAlexaFluor®488
      PresentationPurified mouse monoclonal antibody IgG1 in PBS with 1.5% BSA and 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate, Cat. No. MABN889-AF488, is a highly specific mouse monoclonal antibody that targets Gly-Ala repeats (C9orf72) and has been tested in Immunohistochemistry and Western Blotting.
      Key Applications
      • Immunohistochemistry
      • Western Blotting
      Application NotesImmunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Immunohistochemistry applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).

      Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Western Blotting applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).
      Biological Information
      ImmunogenPolyethylene glycol conjugated linear peptide containng repeat of 10 Gly-Ala repeats.
      Clone5E9
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityClone 5E9 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 based on 100% sequence homology.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Purification MethodProtein G purified
      UniProt Number
      Molecular Weight~54 kDa observed. Uncharacterized bands may be observed in some lysate(s).
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in C9ORF72/C9RANT (poly-GA) GST fusion recombinant 15 GA repeats.

      Western Blotting Analysis: 4 µg/mL of this antibody detected Gly-Ala repeats in GST-fusion recombinant C9orf72 protein.
      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 Size100 µL
      Global Trade Item Number
      Catalogue Number GTIN
      MABN889-AF488 04054839253164