Huntington disease (HD) is a neurodegenerative disorder caused by an expansion of a polyglutamine (PolyQ) domain in the protein huntingtin (htt) that leads to its aggregation into fibrils.
PolyQ expansion above 35 -40 results in disease associated with htt aggregation into inclusion bodies.
These expanded PolyQ repeats adopt multiple potentially toxic conformations increase atrophin-1 and huntingtin levels and abnormally sequester proteins that are essential for transcription.
PolyQ domains of different lengths can display different conformations.
The primary sites of neuropathology show variations between different PolyQ disorders, but usually include the cerebellum, striatum, cerebral cortex, brainstem, spinal cord, and thalamus.
Huntington s disease (HD) and dentatorubral pallidoluysian atrophy (DRPLA) display clinical similarities, but exhibit different regional pathologies.
For example, in HD striatum is the most affected region whereas in DRPLA dentate nucleus of the cerebellum is more severely affected.
052617 binds to the polyQ repeat in htt and is reported to increased htt-induced toxicity and aggregation.
Applications:Suitable for use in Western Blot, Immunocytochemistry and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 0.5ug/ml detected expanded polyQ repeats in Htt in 10 µg of mouse brain tissue lysate.
It does not react with normal Htt
Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4℃ for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20℃.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
抗体種類：Mab
宿主：mouse
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A/G affinity chromatography.
Form：Supplied as a liquid in 0.1M Tris-Glycine, pH 7.4, 150mM sodium chloride, 0.05% sodium azide.
Specificity：Recognizes human ATN1 at the polyQ domain and does not react with wild-type Htt in any significant manner. Species Crossreactivity: mouse and rat
Clone # USB：MW1
Isotype：IgG2b
Calc Applications Abbrev：IC IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：GST-tagged recombinant proteins expressed from two constructs containing the polyQ domain (19 repeats) and 34 amino acids of the dentatorubralpalliodoluysian atrophy (DRPLA) gene.
