Anti-HD-polySer-Ct

Description General description 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. PolySer is a sense repeat-associated non-ATG (RAN) translation protein that accumulates in various brain regions with toxic effects leading to microglial activation and neuronal loss. HD-RAN polySer toxicity is shown to be comparable to that of polyGln. Aggregation of polySer RAN proteins is shown in cells expressing longer repeats greater than 52, which are typically associated with earlier disease onset and in more severe juvenile HD. 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. Specificity This rabbit polyclonal antibody specifically detects RAN proteins with PolySer. Immunogen A synthetic peptides from the C-terminal regions of the predicted 21 polySer frames of HD in the CAG direction. Epitope: unknown Application Anti-HD-polySer-Ct, Cat. No. ABN1673, is a highly specific rabbit polyclonal antibody that targets HD-polySer-Ct and has been tested for use in Immunocytochemistry, Immunohistochemistry, and Western Blotting. Research Category Neuroscience Western Blotting Analysis: A 1:500 dilution from a representative lot detected HD-polySer-Ct in HEK293T cells transfected with HD-polySer-Ct. Western Blotting Analysis: A representative lot detected HD-polySer-Ct in Western Blotting applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77). Immunocytochemistry Analysis: A representative lot detected HD-polySer-Ct in Immunocytochemistry applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77). Immunohistochemistry Analysis: A representative lot detected HD-polySer-Ct in Immunohistochemistry applications (Banez-Coronel, M., et. al. (2015). Neuron. 88(4):667-77). Target description ~27 kDa observed. Uncharacterized bands may be observed in some lysate(s). Physical form Rabbit polyclonal antiserum 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. 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. Quality Evaluated by Western Blotting in HEK293T cells transfected with HD-polySer-Ct. Western Blotting Analysis (WB): A 1:1,000 dilution of this antibody detected PolySer in HEK293T cells transfected with HD-polySer-Ct. Other Notes Concentration: Please refer to lot specific datasheet.