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Lionel M. Igaz et al.
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Mauricio Budini et al.
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Rodent Models of TDP-43 Proteinopathy: Investigating the Mechanisms of TDP-43-Mediated Neurodegeneration
Tania F. Gendron et al.
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Han-Xiang Deng et al.
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Magdalini Polymenidou et al.
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An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity
Weirui Guo et al.
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Iga Wegorzewska et al.
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Dorothee Dormann et al.
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Takashi Nonaka et al.
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Lucia Corrado et al.
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Dorothee Dormann et al.
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R. Pamphlett et al.
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Caroline Vance et al.
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T. J. Kwiatkowski et al.
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TDP-43 mutation in familial amyotrophic lateral sclerosis
Akio Yokoseki et al.
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Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Masato Hasegawa et al.
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Structural determinants of the cellular localization and shuttling of TDP-43
Youhna M. Ayala et al.
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TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology:: a genetic and histopathological analysis
Vivianna M. Van Deerlin et al.
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K Takeda et al.
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