Huntingtin

Huntingtin (HTT) is a soluble 3144 amino acid (348 kDa) protein, with the highest levels of expression being found in the CNS and testes. An abnormal expansion of a glutamine stretch (polyQ) in N-terminal sequence of huntingtin leads to the devastating neurodegenerative disorder Huntington's disease (HD). Subcellular fractionation and microscopic studies have shown that huntingtin is associated with vesicles and microtubules by interacting with huntingtin-associated protein 1 (HAP1), which is reported to form a complex with the dynactin and modulate or regulate the dynein–dynactin complex. There is evidence that huntingtin participates in post-Golgi trafficking of proteins that follow the regulated secretory pathway.

Huntingtin 相关产品（1）

货号	产品名	Cas	产品描述
PL03993	TPE-MI	1245606-71-6	TPE-MI (Tetraphenylethene maleimide) 在通过马来酰亚胺与硫醇结合之前本质上是非荧光的。TPE-MI 荧光在标记游离半胱氨酸硫醇时被激活，该硫醇通常埋在展开时暴露的球状蛋白质的核心中。TPE-MI 可用于测量细胞未折叠蛋白质负荷。TPE-MI 可以报告亨廷顿病诱导多能干细胞模型中的蛋白质平衡失衡，以及在形成可见聚集物之前转染突变亨廷顿外显子1的细胞。TPE-MI 还检测双氢青蒿素治疗疟疾寄生虫后的蛋白质损伤。