Article
Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification.
Brain research. Molecular brain research - 27 Apr 2005
Watanabe Yasuhiro, Yasui Kenichi, Nakano Toshiya, Doi Koji, Fukada Yasuyo, Kitayama Michio, Ishimoto Miho, Kurihara Saiko, Kawashima Mika, Fukuda Hiroki, Adachi Yoshiki, Inoue Takao, Nakashima Kenji
Abstract excerpt
Mutation of Cu/Zn superoxide dismutase (SOD1) contributes to a portion of the cases of familial amyotrophic lateral sclerosis (FALS). We previously reported on a FALS family whose members had a mutant form of SOD1 characterized by a 2-base pair (bp) deletion at codon 126 of the SOD1 gene. To investigate the cellular consequences of this mutation, we produced transgenic mice that expressed normal and mutated...
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