MABN784

Anti-FMR1polyG Antibody, clone 2J7

clone 2J7, from mouse

• Synonym(s): Fragile X mental retardation protein 1 RANT fusion, FMR1polyG, FMRP RANT fusion, FMR-1 RANT fusion
• UNSPSC Code: 12352203
• eCl@ss: 32160702

Properties

• biological source: mouse
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: 2J7, monoclonal
• species reactivity: human
• technique(s): immunocytochemistry: suitable; immunohistochemistry: suitable; western blot: suitable
• isotype: IgG2bκ
• UniProt accession no.: Q06787
• shipped in: wet ice
• target post-translational modification: unmodified
• Gene Information: human ... FRAXA(2477) , FRAXA(108684022)

Description

General description

Fragile 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.

Specificity

This 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.

Immunogen

Epitope: C-terminal region.

Ovalbumin-conjugated linear peptide corresponding to a sequence from the C-terminal region of FMR1polyG.

Application

Immunohistochemistry 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).

Research Category
Neuroscience

Research Sub Category
Neurodegenerative Diseases

Quality

Evaluated 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.

Target description

Variable, depending on the sizes of the FMR1polyG fusions and aggregates.

Physical form

Format: Purified

Protein G Purified

Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

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.

Safety Information

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: WGK 1
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable