Concepedia

Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein

Hitoshi Aizawa, Takenari Yamashita, Haruhisa Kato, Takashi Kimura, Shin Kwak

Journal of Clinical Neurology · 2019 · 38 citations · 16 references

DOIFull text

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Abstract

Our findings suggest a close relationship between NPC alterations and TDP-43 pathology in the degenerative process of the motor neurons of sALS patients.

References

16

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

Manuela Neumann, Deepak M. Sampathu, Linda K. Kwong et al. · Science · 2006 · 6.5K citations

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

Tetsuaki Arai, Masato Hasegawa, Haruhiko Akiyama et al. · Biochemical and Biophysical Research Communications · 2006 · 2.7K citations

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The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

Ke Zhang, Christopher J. Donnelly, Aaron R. Haeusler et al. · Nature · 2015 · 974 citations

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GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

Brian D. Freibaum, Yubing Lu, Nam Chul Kim et al. · Nature · 2015 · 831 citations · Full text

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Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

Ana Jovičić, Jérôme Mertens, Steven Boeynaems et al. · Nature Neuroscience · 2015 · 616 citations · Full text

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