Article
pS421 huntingtin modulates mitochondrial phenotypes and confers neuroprotection in an HD hiPSC model.
Cell death & disease - 25 Sept 2020
Xu Xiaohong, Ng Bryan, Sim Bernice, Radulescu Carola I, Yusof Nur Amirah Binte Mohammad, Goh Wah Ing, Lin Shuping, Lim John Soon Yew, Cha Yoonjeong, Kusko Rebecca, Kay Chris, Ratovitski Tamara, Ross Christopher, Hayden Michael R, Wright Graham, Pouladi Mahmoud A
Abstract excerpt
Huntington disease (HD) is a hereditary neurodegenerative disorder caused by mutant huntingtin (mHTT). Phosphorylation at serine-421 (pS421) of mHTT has been shown to be neuroprotective in cellular and rodent models. However, the genetic context of these models differs from that of HD patients. Here we employed human pluripotent stem cells (hiPSCs), which express endogenous full-length mHTT. Using genome editing,...
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