MABN1788

Anti-FMR1polyG Antibody, clone 9FM-1B7

ascites fluid, clone 9FM-1B7, from mouse

• Synonym(s): 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
• UNSPSC Code: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

Properties

• biological source: mouse
• Quality Level: 100
• antibody form: ascites fluid
• antibody product type: primary antibodies
• clone: 9FM-1B7, monoclonal
• species reactivity: human
• technique(s): immunohistochemistry: suitable; western blot: suitable
• isotype: IgG1κ
• UniProt accession no.: Q06787
• shipped in: dry 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

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

Immunohistochemistry 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).

Research Category
Neuroscience

Research Sub Category
Neurodegenerative Diseases

Quality

Evaluated 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.

Target description

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

Physical form

Mouse monoclonal IgG1κ antibody in ascites with 0.05% sodium azide.

Unpurified

Storage and Stability

Stable 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.

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