Nuclear pore destruction and loss of nuclear TDP-43 in FUS mutation-related amyotrophic lateral sclerosis motor neurons

J Neurol Sci. 2022 May 15:436:120187. doi: 10.1016/j.jns.2022.120187. Epub 2022 Feb 16.

Authors

Hitoshi Aizawa¹, Sayaka Teramoto², Takuto Hideyama², Haruhisa Kato², Hiroo Terashi², Yasuhiro Suzuki³, Takashi Kimura³, Shin Kwak²

Affiliations

¹ Department of Neurology, Tokyo Medical University, Japan. Electronic address: haizawa@tokyo-med.ac.jp.
² Department of Neurology, Tokyo Medical University, Japan.
³ Department of Neurology, Asahikawa Medical Center, National Hospital Organization, Japan.

PMID: 35232585
DOI: 10.1016/j.jns.2022.120187

No abstract available

Keywords: Amyotrophic lateral sclerosis; Fused in sarcoma; Nuclear pore complex; Nucleoporin; TDP-43.

Publication types

Letter

MeSH terms

Amyotrophic Lateral Sclerosis* / genetics
DNA-Binding Proteins / genetics
DNA-Binding Proteins / metabolism
Humans
Motor Neurons
Mutation / genetics
Nuclear Pore / metabolism
RNA-Binding Protein FUS / genetics

Substances

DNA-Binding Proteins
FUS protein, human
RNA-Binding Protein FUS