RAN, a small GTP binding protein, belonging to the RAS superfamily is involved in the translocation of RNA and proteins through the nuclear pore complex and participate in the control of DNA synthesis and cell cycle progression. Antisense QAGR (Gln-Ala-Gly-Arg ) RAN proteins that are localized primarily in the nucleus of oligodendrocytes are reported to accumulate within the white matter region of the brain. They are detectable in frontal cortex, basal ganglia, and hippocampus in subjects with myotonic dystrophy type 2 (DM2) but are not detected in normal control brain tissue. QAGR proteins are known to be toxic to cells independent of RNA effects. QAGR RAN proteins also accumulate in regions of brain that display certain neuropathological changes. These include regions with accumulation of activated microglia, increased astrocytes, and white matter rarefaction. Studies have shown that the tetranucleotide DM2 CAGG expansion mutation is bidirectionally transcribed and the resulting RNAs are RAN translated and produce tetrapeptide expansion proteins with QAGR repeats from the antisense strand, which accumulate in brains from DM2 subjects. (Ref.: Zu, T., et al. (2017). Neuron 95 (8); 1292-1305).

~26 kDa observed. Uncharacterized bands may be observed in some lysate(s).

This rabbit polyclonal antibody specifically detects proteins with poly-(QAGR).

A synthetic peptide corresponding to the (QAGR)EXP repeat motif.

Epitope: unknown

Anti-poly-(QAGR), Cat. No. ABN2271, is a highly specific rabbit polyclonal antibody that targets poly-(QAGR) and has been tested for use in Immunohistochemistry, Immunofluorescence, and Western Blotting.

Research Category
Neuroscience

Western Blotting Analysis: A representative lot detected poly-(QAGR) in Western Blotting applications (

Immunofluorescence Analysis: A representative lot detected poly-(QAGR) in Immunofluorescence applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77).

Immunohistochemistry Analysis: A representative lot detected poly-(QAGR) in Immunohistochemistry applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77).

Rabbit polyclonal antiserum with 0.05% sodium azide

Unpurified

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.

Evaluated by Western Blotting in HEK293Tcells transfected with poly-(QAGR).


Western Blotting Analysis (WB): A 1:1,000 dilution of this antibody detected poly-(QAGR) in HEK293T cells transfected with poly-(QAGR), but not in control cells.

Concentration: Please refer to lot specific datasheet.

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.