Article
4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells.
Stem cells (Dayton, Ohio) - 1 Jun 2016
Naujock Maximilian, Stanslowsky Nancy, Bufler Sebastian, Naumann Marcel, Reinhardt Peter, Sterneckert Jared, Kefalakes Ekaterini, Kassebaum Carola, Bursch Franziska, Lojewski Xenia, Storch Alexander, Frickenhaus Marie, Boeckers Tobias M, Putz Stefan, Demestre Maria, Liebau Stefan, Klingenstein Moritz, Ludolph Albert C, Dengler Reinhard, Kim Kwang-Soo, Hermann Andreas, Wegner Florian, Petri Susanne
Abstract excerpt
Despite decades of research on amyotrophic lateral sclerosis (ALS), there is only one approved drug, which minimally extends patient survival. Here, we investigated pathophysiological mechanisms underlying ALS using motor neurons (MNs) differentiated from induced pluripotent stem cells (iPSCs) derived from ALS patients carrying mutations in FUS or SOD1. Patient-derived MNs were less active and excitable compared...
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