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ABN1644 Anti-C9orf72 (short form)

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ABN1644
100 μg  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IP, IF, IHCRbPurifiedPolyclonal Antibody
      Description
      Catalogue NumberABN1644
      DescriptionAnti-C9orf72 (short form)
      Alternate Names
      • C9-S
      • C9orf72 short isofrom
      • Protein C9orf72 Short isoform
      Background InformationProtein C9orf72 (UniProt: Q96LT7) is encoded by the C9orf72 gene (Gene ID: 203228) in human. Expansion of a GGGGCC (G4C2) hexanucleotide repeat sequence in the non-coding region of human chromosome 9 open reading frame 72 or C9orf72 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 majority of normal human C9orf72 alleles carry less than 20 repeats, whereas large expansions consisting of hundreds or thousands of repeats lead to disease. C9orf72 generates three transcripts through alternative splicing that encode 2 protein isoforms; a long isoform of about 54 kDa (termed C9-L), corresponding to variants 2 and 3, and a short isoform of about 24 kDa (termed C9-S) corresponding to variant 1. Both isoforms are widely expressed, including kidney, lung, liver, heart, testis and several brain regions, including cerebellum, and frontal cortex. Both, C9-L and C9-S are reported to display distinct subcellular localizations in cerebellar Purkinje cells of both c9-ALS and nonc9-ALS cases. C9-L is shown to be spread diffusely throughout the cytoplasm, whereas C9- S is localized to the nuclear membrane of Purkinje cells of both c9-ALS and non-c9-ALS cases. Also, C9-L is shown to be present mainly in the high-salt Triton and urea-soluble fractions, whereas C9-S is shown to be abundant in both the low- and high-salt Triton fractions, indicating differences in their subcellular localizations. Antibodies generated against C9-L and C9-S offer advantage because they allow characterization of different biochemical profiles, region-specific changes, and subcellular localizations that is not possible with antibodies generated against the common N-terminal region.
      References
      Product Information
      FormatPurified
      PresentationPurified rabbit polyclonal antibody in PBS with 0.02% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-C9ORF72 (short form), Cat. No. ABN1644, is a highly specific rabbit polyclonal antibody that targets short isoform of C9orf72 protein and has been tested in Immunofluorescence, Immunohistochemistry, Immunoprecipitation, and Western Blotting.
      Key Applications
      • Western Blotting
      • Immunoprecipitation
      • Immunofluorescence
      • Immunohistochemistry
      Application NotesImmunoprecipitation Analysis: A representative lot detected C9ORF72 (short form) in N2a cells (Xiao, S., et. al. (2015). Ann Neurol. 78(4):568-83).

      Western Blotting Analysis: A representative lot detected C9ORF72 (short form) in different regions of the central nervous system (Xiao, S., et. al. (2015). Ann Neurol. 78(4):568-83).

      Immunohistochemistry Analysis: A representative lot detected C9ORF72 (short form) in spinal motor neurons (Xiao, S., et. al. (2015). Ann Neurol. 78(4):568-83).

      Immunofluorescence Analysis: A representative lot detected C9ORF72 (short form) in lumbar spinal cord tissue (Xiao, S., et. al. (2015). Ann Neurol. 78(4):568-83).
      Biological Information
      ImmunogenKLH-conjugated linear peptide corresponding to 12 amino acids from the C-terminal of short isoform (C9-S) of human C9orf72 with terminal asparagine substituted with lysine.
      EpitopeC-terminus
      ConcentrationPlease refer to lot specific datasheet.
      HostRabbit
      SpecificityThis polyclonal antibody specifically detects the short isoform (C9-S) of C9orf72 protein. It targets an epitope within 12 amino acids from the C-terminal region.
      IsotypeIgG
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • C9orf72
      Purification MethodAffinity Purified
      UniProt Number
      Molecular Weight~25 kDa observed; 24.76 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 human cerebellum tissue lysate.

      Western Blotting Analysis: A 1:500 dilution of this antibody detected C9ORF72 (short form) in 10 µg of human cerebellum tissue 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 2-8°C from date of receipt.
      Packaging Information
      Material Size100 μg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      ABN1644 04054839189111