Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions

Article ID	Journal	Published Year	Pages	File Type
8684529	Experimental Neurology	2018	12 Pages	PDF

Abstract

Our observation suggested that TDP-43 aggregates might not be sufficient to trigger the pathogenesis of sIBM although myofiber sarcoplasmic aggregation of TDP-43 led to myofiber degeneration via ER stress and possibly calcium dysregulation, independently of inflammatory process.

Keywords

TDP-43 FTLD SIBM MTS MGT DAPI Aβ GAPDH 4′,6-diamidino-2-phenylindole BiP AST Aspartate aminotransferase amyloid β Endoplasmic reticulum stress amyotrophic lateral sclerosis ALS frontotemporal lobar degeneration Sarcoplasmic reticulum endoplasmic reticulum Rimmed vacuole lactate dehydrogenase LDH Sporadic inclusion body myositis hematoxylin-eosin Calcium homeostasis polymerase chain reaction PCR Ubiquitin proteasome Creatine kinase glyceraldehyde-3-phosphate dehydrogenase UPS

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Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions

Authors

Nozomu Tawara, Satoshi Yamashita, Kensuke Kawakami, Takashi Kurashige, Ziwei Zhang, Masayoshi Tasaki, Yasuhiro Yamamoto, Tomo Nishikami, Tsukasa Doki, Xiao Zhang, Yoshimasa Matsuo, En Kimura, Akie Tawara, Yasushi Maeda, Stephen D. Hauschka,