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MABN1788 Anti-FMR1polyG Antibody, clone 9FM-1B7

Anti-FMR1polyG Antibody, clone 9FM-1B7 is an antibody against FMR1polyG for use in Western Blotting, Immunohistochemistry.

Anti-FMR1polyG Antibody, clone 9FM-1B7 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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MABN1788
100 µL  
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Key Spec Table

Species ReactivityKey ApplicationsHostFormatAntibody Type
HWB, IHCMAscitesMonoclonal Antibody
Description
Catalogue NumberMABN1788
DescriptionAnti-FMR1polyG Antibody, clone 9FM-1B7
Alternate Names
  • FMR1polyG
  • FMRpolyG
  • FMR1 RANT
  • FMR1 repeat-associated non-ATG-initiated translation
  • FMR1 repeat-associated non-AUG-initiated translation
  • Fragile X mental retardation protein 1 gene RANT
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.
References
Product Information
FormatAscites
PresentationMouse monoclonal IgG1κ antibody in ascites with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-FMR1polyG Antibody, clone 9FM-1B7 is an antibody against FMR1polyG for use in Western Blotting, Immunohistochemistry.
Key Applications
  • Western Blotting
  • Immunohistochemistry
Application NotesImmunohistochemistry Analysis: A representative lot detected FMR1polyG immunoreactivity in paraffin-embedded brain tissue sections from an Fragile X-associated Tremor/Ataxia syndrome (FXTAS) patient, but not in brain tissues from non-FXTAS individuals (Buijsen, R.A., et al. (2014). Acta Neuropathol. Commun. 2:162).
Western Blotting Analysis: A representative lot detected an exogenously expressed FMR1polyG EGFP fusion construct corresponding to 5′UTR of FMR1 with 50 CGG repeats (Buijsen, R.A., et al. (2014). Acta Neuropathol. Commun. 2:162).
Biological Information
ImmunogenOvalbumin-conjugated linear peptide corresponding to a sequence from the C-terminal region of FMR1polyG.
EpitopeC-terminal region.
Clone9FM-1B7
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.
IsotypeIgG1κ
Species Reactivity
  • Human
Antibody TypeMonoclonal Antibody
Gene Symbol
  • FMR1
  • FRAXA
  • POF1
Purification MethodUnpurified
UniProt Number
Molecular WeightVariable, depending on the sizes of the FMR1polyG fusions and aggregates.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated by Western Blotting in GFP-FMR1polyG-expressing COS cell lysate.

Western Blotting Analysis: A 1:2,000 dilution 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 -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
MABN1788 04055977312430

Documentation

Anti-FMR1polyG Antibody, clone 9FM-1B7 SDS

Title

Safety Data Sheet (SDS) 

Anti-FMR1polyG Antibody, clone 9FM-1B7 Certificates of Analysis

TitleLot Number
Anti-FMR1polyG, clone 9FM-1B7 - 3180868 3180868
Anti-FMR1polyG, clone 9FM-1B7 - 3286828 3286828
Anti-FMR1polyG, clone 9FM-1B7 - 3733772 3733772
Anti-FMR1polyG, clone 9FM-1B7 - 3984017 3984017
Anti-FMR1polyG, clone 9FM-1B7 - 4052771 4052771
Anti-FMR1polyG, clone 9FM-1B7 - 4079398 4079398
Anti-FMR1polyG, clone 9FM-1B7 - 4151185 4151185
Anti-FMR1polyG, clone 9FM-1B7 - 4193022 4193022
Anti-FMR1polyG, clone 9FM-1B7 -Q2638538 Q2638538