Ontology highlight
ABSTRACT:
SUBMITTER: Wainger BJ
PROVIDER: S-EPMC4023477 | biostudies-literature | 2014 Apr
REPOSITORIES: biostudies-literature
Wainger Brian J BJ Kiskinis Evangelos E Mellin Cassidy C Wiskow Ole O Han Steve S W SS Sandoe Jackson J Perez Numa P NP Williams Luis A LA Lee Seungkyu S Boulting Gabriella G Berry James D JD Brown Robert H RH Cudkowicz Merit E ME Bean Bruce P BP Eggan Kevin K Woolf Clifford J CJ
Cell reports 20140403 1
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of the motor nervous system. We show using multielectrode array and patch-clamp recordings that hyperexcitability detected by clinical neurophysiological studies of ALS patients is recapitulated in induced pluripotent stem cell-derived motor neurons from ALS patients harboring superoxide dismutase 1 (SOD1), C9orf72, and fused-in-sarcoma mutations. Motor neurons produced from a genetically corrected but otherwise isogenic SO ...[more]