Huntington disease (HD) is a progressive neurodegenerative disorder is caused by a CAG,CTG expansion in the HTT gene that results in the production of a mutant huntingtin protein (HTT) with polymeric expansions that accumulate in human brain with HD. PolyAla is a sense repeat-associated non-ATG (RAN) translation protein that accumulates in various brain regions with some toxic effects leading to microglial activation and neuronal loss. HD-RAN polyAla toxicity is shown to be lower compared to that of polyGln. The transfection of cells with polyAla construct can express high polyAla levels, but have lower toxicity. HD-polyAla RAN proteins are reported to accumulate in cells expressing CAG repeats that are equal to or greater than 52 but not at repeats less than 45. HD-RAN proteins are abundant in regions of the brain that are most affected by HD and these regions display pathologic features of HD, such as caspase-3 activity and microglial activation. HD-Ran protein accumulation and aggregation in HD brains are shown to be length dependent.

~15 kDa observed. Uncharacterized bands may be observed in some lysate(s).

A synthetic peptides from the C-terminal regions of the predicted 21 polyAla frame of HD in the CAG direction.

Anti-HD-polyAla Ct, Cat. No. ABN1672, is a highly specific rabbit polyclonal antibody that targets HD-polyAla Ct and has been tested in Immunocytochemistry, Immunohistochemistry, and Western Blotting.

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

Western Blotting Analysis: A representative lot detected HD-polyAla Ct in Western Blotting applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77).

Immunocytochemistry Analysis: A representative lot detected HD-polyAla Ct in Immunocytochemistry applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77).

Western Blotting Analysis: A 1:1,000 dilution from a representative lot detected HD-polyAla Ct in pcDNA3.1 (Ctrl) & HEK293T-HD-polyAla-Ct.

Research Category
Neuroscience

This rabbit polyclonal antibody specifically detects RAN proteins with PolyAla.

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 HEK293T cells transfected with HD-polyAla-Ct.

Western Blotting Analysis (WB): A 1:1,000 dilution of this antibody detected PolyAla in HEK293T cells transfected with HD-polyAla-Ct.

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.