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.
This rabbit polyclonal antibody specifically detects RAN proteins with PolyAla.
A synthetic peptides from the C-terminal regions of the predicted 21 polyAla frame of HD in the CAG direction.
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.
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.
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.
~15 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Rabbit polyclonal antiserum with 0.05% sodium azide.
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.
Concentration: Please refer to lot specific datasheet.
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