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MABN784 Anti-FMR1polyG Antibody, clone 2J7

Anti-FMR1polyG Antibody, clone 2J7 MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Neurodegenerative Diseases Gene Symbol: FMR1, FRAXA, POF1 UniProt Number: Q06787
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MABN784
100 μg  
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      Overview

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      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IHC, ICCMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABN784
      DescriptionAnti-FMR1polyG Antibody, clone 2J7
      Alternate Names
      • Fragile X mental retardation protein 1 RANT fusion
      • FMR1polyG
      • FMRP RANT fusion
      • FMR-1 RANT fusion
      Background InformationFragile X-associated Tremor/Ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by an elevated CGG-repeat expansions (55-200) in the 5′ UTR of the fragile-X mental retardation 1 gene FMR1 (also known as FRAXA, POF1; Gene ID 2332) on the X-chromosome. The CGG repeat expansion in FXTAS triggers repeat-associated non-ATG-initiated translation (RAN or RANT), also known as repeat-associated non-AUG-initiated translation, within the 5’UTR of FMR1 mRNA, resulting in the production of fusion proteins (FMR1polyG) that contain N-terminal polyglycine fused to either C-terminal FMR1 in-frame sequence or one of two FMR1 frame-shift sequences. Immunohistochemical analysis of FXTAS brain tissues reveals FMR1polyG-positive aggregates that closely resemble neuronal intranuclear inclusions seen in polyglutamine diseases and other protein-mediated neurodegenerative disorders. Much larger expansions of the same repeat cause fragile X syndrome (FRAX), the most common inherited form of mental retardation, by silencing FMR1 transcription.
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      • Immunocytochemistry
      Application NotesImmunohistochemistry Analysis: A representative lot detected FMR1polyG immunoreactivity in paraffin-embedded brain tissue sections from Fragile X-associated Tremor/Ataxia syndrome (FXTAS) patients, but not in brain tissues from non-FXTAS individuals (Todd, P.K., et al. (2013). Neuron. 78(3):440-455).
      Immunohistochemistry Analysis: A representative lot detected both ubiquitinated inclusions-associated and non-inclusions-associated FMR1polyG immunoreactivity by dual fluorescent immunohistochemistry using paraffin-embedded brain tissue sections from Fragile X-associated Tremor/Ataxia syndrome (FXTAS) patients (Todd, P.K., et al. (2013). Neuron. 78(3):440-455).
      Western Blotting Analysis: A representative lot detected recombinant FMRpolyG GST fusion constructs, while only one immunoreactive band (~15 kDa) could be detected in Fragile X-associated Tremor/Ataxia syndrome (FXTAS) patients brain lysates due to extended aggregation of FMR1polyG (Todd, P.K., et al. (2013). Neuron. 78(3):440-455).
      Immunocytochemistry Analysis: A representative lot detected exogenously expressed FLAG-FMR1polyG constructs by fluorescent immunocytochemistry (Todd, P.K., et al. (2013). Neuron. 78(3):440-455).
      Biological Information
      ImmunogenOvalbumin-conjugated linear peptide corresponding to a sequence from the C-terminal region of FMR1polyG.
      EpitopeC-terminal region.
      Clone2J7
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityThis monoclonal antibody does not detect the Fragile X mental retardation protein 1 sequences reported by UniProt (Q06787-1 through Q06787-9). It detects one of the two possible types of FMR1 frame-shift sequences with N-terminal polyG fusion found in Fragile X-associated Tremor/Ataxia syndrome (FXTAS) patients due to CGG repeats expansion (55-200 repeats) in the 5’UTR of the FMR1 gene. Due to the extended aggregation of the pathological forms of FMR1polyG, the detection of FMR1polyG in patient samples by Western blotting is not recommended.
      IsotypeIgG2bκ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Gene Symbol
      • FMR1
      • FRAXA
      • POF1
      Purification MethodProtein G Purified
      UniProt Number
      Molecular WeightVariable, depending on the sizes of the FMR1polyG fusions and aggregates.
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in COS GFP-FMRpolyG cells.

      Western Blotting Analysis: 1.0 µg/mL of this antibody detected an exogenously expressed FMR1polyG GFP fusion construct in transfected COS cells.
      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
      Global Trade Item Number
      Catalogue Number GTIN
      MABN784 04055977311846