1. Signaling Pathways
  2. Neuronal Signaling
  3. Huntingtin

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 (4):

目录号 产品名 作用方式 纯度
  • HY-143218
    TPE-MI 98.54%
    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。
  • HY-132579
    Tominersen
    Tominersen (RG6042) 是第二代 2′-O-(2-甲氧基乙基) 反义寡核苷酸,靶向亨廷蛋白 (HTT) mRNA,有效抑制 HTT 的合成。Tominersen 能在小鼠中提高存活率并减少脑萎缩。Tominersen 可用于亨廷顿病 (HD) 的研究。
  • HY-132593
    Rovanersen
    Rovanersen (WVE-120101) 是一种反义寡核苷酸,通过特异靶向 huntingtin (HTT) 基因的突变mRNA拷贝,而不影响正常的 HTT基因的mRNA,从而防止有缺陷的 huntingtin 蛋白的产生。Rovanersen 可用于亨廷顿病的研究。
  • HY-132594
    Lexanersen
    Lexanersen (WVE-120102) 是可用于亨廷顿舞蹈症研究的寡核苷酸。