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The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

Title
The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies
Authors
Jo, MyungjinLee, ShinryeJeon, Yu-MiKim, SeyeonKwon, YounghwiKim, Hyung-Jun
DGIST Authors
Jo, Myungjin; Lee, Shinrye; Jeon, Yu-Mi; Kim, Seyeon; Kwon, Younghwi; Kim, Hyung-Jun
Issue Date
2020-10
Citation
Experimental and Molecular Medicine, 52(10), 1652-1662
Type
Article
Article Type
Review
Keywords
MUTATIONSPRIONSPTDP-43 PATHOLOGYNUCLEAR IMPORTFRONTOTEMPORAL LOBAR DEGENERATIONAMYOTROPHIC-LATERAL-SCLEROSISTAR-DNA-BINDINGALZHEIMERS-DISEASEALPHA-SYNUCLEINAGGREGATION
ISSN
1226-3613
Abstract
TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 aggregates in the central nervous system is a common feature of many neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Alzheimer’s disease (AD), and limbic predominant age-related TDP-43 encephalopathy (LATE). Accumulating evidence suggests that prion-like spreading of aberrant protein aggregates composed of tau, amyloid-β, and α-synuclein is involved in the progression of neurodegenerative diseases such as AD and PD. Similar to those of prion-like proteins, pathological aggregates of TDP-43 can be transferred from cell-to-cell in a seed-dependent and self-templating manner. Here, we review clinical and experimental studies supporting the prion-like spreading of misfolded TDP-43 and discuss the molecular mechanisms underlying the propagation of these pathological aggregated proteins. The idea that misfolded TDP-43 spreads in a prion-like manner between cells may guide novel therapeutic strategies for TDP-43-associated neurodegenerative diseases. © 2020, The Author(s).
URI
http://hdl.handle.net/20.500.11750/12795
DOI
10.1038/s12276-020-00513-7
Publisher
Springer Nature
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