Protein transmission in neurodegenerative disease

C Peng, JQ Trojanowski, VMY Lee - Nature Reviews Neurology, 2020 - nature.com
Most neurodegenerative diseases are characterized by the intracellular or extracellular
aggregation of misfolded proteins such as amyloid-β and tau in Alzheimer disease, α …

Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

Structure of pathological TDP-43 filaments from ALS with FTLD

D Arseni, M Hasegawa, AG Murzin, F Kametani, M Arai… - Nature, 2022 - nature.com
The abnormal aggregation of TAR DNA-binding protein 43 kDa (TDP-43) in neurons and
glia is the defining pathological hallmark of the neurodegenerative disease amyotrophic …

Development of multifunctional molecules as potential therapeutic candidates for Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis in the …

MG Savelieff, G Nam, J Kang, HJ Lee, M Lee… - Chemical …, 2018 - ACS Publications
Neurodegenerative diseases pose a substantial socioeconomic burden on society.
Unfortunately, the aging world population and lack of effective cures foreshadow a negative …

The role of liquid–liquid phase separation in aggregation of the TDP-43 low-complexity domain

WM Babinchak, R Haider, BK Dumm, P Sarkar… - Journal of Biological …, 2019 - ASBMB
Pathological aggregation of the transactive response DNA-binding protein of 43 kDa (TDP-
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …

Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage

ST Kumar, S Nazarov, S Porta, N Maharjan… - Nature …, 2023 - nature.com
Despite the strong evidence linking the transactive response DNA-binding protein 43 (TDP-
43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43 …

Spreading of pathology in neurodegenerative diseases: a focus on human studies

J Brettschneider, KD Tredici, VMY Lee… - Nature Reviews …, 2015 - nature.com
The progression of many neurodegenerative diseases is thought to be driven by the
template-directed misfolding, seeded aggregation and cell–cell transmission of …

Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins

A Ratti, E Buratti - Journal of neurochemistry, 2016 - Wiley Online Library
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

JL Guo, VMY Lee - Nature medicine, 2014 - nature.com
A common feature of many neurodegenerative diseases is the deposition of β-sheet-rich
amyloid aggregates formed by proteins specific to these diseases. These protein aggregates …

Protein aggregation in amyotrophic lateral sclerosis

AM Blokhuis, EJN Groen, M Koppers… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …