Merck Anti-polyQ specific Antibody, clone MW1

Anti-polyQ specific Antibody, clone MW1

clone MW1, from mouse

Htt mutant, PolyQ-Htt, Clone MW1, Atrophin-1

Quality Level

100

생물학적 소스

mouse

항체 형태

purified immunoglobulin

antibody product type

primary antibodies

클론

MW1, monoclonal

species reactivity

mouse, rat, human

포장

antibody small pack of 25 μg

technique(s)

immunocytochemistry: suitable
immunohistochemistry: suitable
western blot: suitable

동형

IgG2b

NCBI 수납 번호

NP_001007027

UniProt 수납 번호

P54259

배송 상태

ambient

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. Clone MW1 strongly binds to the expanded PolyQ of mutant Htt, but does not display any detectible binding to normal human or wild-type murine Htt. The clone MW1, which bind to the polyQ repeat in htt is reported to increase htt-induced toxicity and aggregation. (The clone MW1, which bind to the polyQ repeat in htt is reported to increased htt-induced toxicity and aggregation. (Ref.: Luthi-Carter, R et al. (2012). Hum. Mol. Gen. 11(17); 1927-1937; Legleiter, J et al. (2009). J. Biol. Chem. 284(32); 21647-21658; Ko, J et al. (2001). Brain Res. Bull. 56 (3/4); 319-329).