Article
Knockdown of genes involved in axonal transport enhances the toxicity of human neuromuscular disease-linked MATR3 mutations in Drosophila.
FEBS letters - 1 Sept 2020
Zhao Melody, Kao Ching Serena, Arndt Claudia, Tran David Duc, Cho Woo In, Maksimovic Katarina, Chen Xiao Xiao Lily, Khan Mashiat, Zhu Hongxian, Qiao Julia, Peng Kailong, Hong Jingyao, Xu Jialu, Kim Deanna, Kim Jihye Rachel, Lee Jooyun, van Bruggen Rebekah, Yoon Wan Hee, Park Jeehye
Abstract excerpt
Mutations in the nuclear matrix protein Matrin 3 (MATR3) have been identified in amyotrophic lateral sclerosis and myopathy. To investigate the mechanisms underlying MATR3 mutations in neuromuscular diseases and efficiently screen for modifiers of MATR3 toxicity, we generated transgenic MATR3 flies. Our findings indicate that expression of wild-type or mutant MATR3 in motor neurons reduces climbing ability and...
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