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ABN1354 Anti-C9ORF72/C9RANT (Poly-PR)

Anti-C9ORF72/C9RANT (Poly-PR), Cat. No. ABN1354, is a highly specific rabbit polyclonal antibody, that targets C9ORF72 (alpha-PR antisense) and has been tested in Dot Blot, Immunofluorescence, Immunohistochemistry, and Western Blotting.

Anti-C9ORF72/C9RANT (Poly-PR) MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Gene Symbol: C9orf72 UniProt Number: Q96LT7
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ABN1354
100 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HIF, IHC, DB, WBRbSerumPolyclonal Antibody
      Description
      Catalogue NumberABN1354
      DescriptionAnti-C9ORF72/C9RANT (Poly-PR)
      Alternate Names
      • C9ORF72/C9RANT (Poly-PR)
      • alpha-PR antisense
      • C9ORF72 (Poly-RP)
      Background InformationExpansion of a GGGGCC (G4C2) 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 from both sense and antisense transcripts generates five types of dipeptide repeat (DPR) protein species, each composed of repeating units of two amino acids, glycine-alanine (GA), glycine-proline (GP), glycine-arginine (GR), alanine-proline (AP), proline-arginine (PR), respectively. The sense transcript (5'-GGGGCC-3' hexanucleotide repeats) is translated through repeat-associated non-ATG (RAN) translation such that poly- (Gly- Ala), poly-(Gly-Pro), and poly-(Gly-Arg) proteins were produced. The anti-sense transcript (containing 5'-GGCCCC-3' (G2C4) hexanucleotide repeats is translated through repeat-associated non-ATG (RAN) translation producing poly-(Pro-Ala), poly- (Pro- Arg), poly- (Gly- Pro) proteins. The antisense proteins accumulate in cytoplasmic aggregates in affected brain regions, including the frontal and motor cortex, hippocampus, and spinal cord neurons.
      References
      Product Information
      FormatSerum
      PresentationRabbit polyclonal serum with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-C9ORF72/C9RANT (Poly-PR), Cat. No. ABN1354, is a highly specific rabbit polyclonal antibody, that targets C9ORF72 (alpha-PR antisense) and has been tested in Dot Blot, Immunofluorescence, Immunohistochemistry, and Western Blotting.
      Key Applications
      • Immunofluorescence
      • Immunohistochemistry
      • Dot Blot
      • Western Blotting
      Application NotesImmunofluorescence Analysis: A 1:500 dilution from a representative lot detected C9ORF72/C9RANT (Poly-PR) in HEK293Transfected Flag-PR. (Courtesy of Dr. Laura P.W. Ranum and Dr. Lien Nguyen from the University of Florida).

      Immunofluorescence Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in transfected HEK293T Flag-PA cells (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).


      Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in recombinant Protein (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).

      Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (Poly-PR) in aggregates found in neurons of the CA and DG regions of the hippocampus (Zu, T., et. al. (2013). Proc. Natl. Acad. Sci. USA. 110(51):E4968-77).

      Dot Blotting Analysis: A representative lot detected C9ORF72/C9RANT (PR-CT) in Dot Blotting applications (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).
      Biological Information
      ImmunogenAc-RPRPRPRPRPRPRPRPRC-amide.
      Epitopeunknown
      ConcentrationPlease refer to lot specific datasheet.
      HostRabbit
      SpecificityThis polyclonal antibody recognizes C9ORF72/C9RANT (poly-PR).
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • C9orf72
      Purification MethodUnpurified
      UniProt Number
      Molecular Weight~18 kDa observed. 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 lysate from Flag-PR transfected HEK293 cells.

      Western Blotting Analysis: A 1:1,000 dilution of this antibody detected C9ORF72/C9RANT (Poly-PR) in 10 µL of lysate from HEK293 cells transfected with Flag-PR.
      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
      Catalogue Number GTIN
      ABN1354 04054839167430