The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …

[HTML][HTML] 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 …

[HTML][HTML] The integral role of RNA in stress granule formation and function

D Campos-Melo, ZCE Hawley… - Frontiers in Cell and …, 2021 - frontiersin.org
Stress granules (SGs) are phase-separated, membraneless, cytoplasmic ribonucleoprotein
(RNP) assemblies whose primary function is to promote cell survival by condensing …

RNA-binding proteins with prion-like domains in health and disease

AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …

Cryo-EM structures of four polymorphic TDP-43 amyloid cores

Q Cao, DR Boyer, MR Sawaya, P Ge… - Nature structural & …, 2019 - nature.com
The DNA and RNA processing protein TDP-43 undergoes both functional and pathogenic
aggregation. Functional TDP-43 aggregates form reversible, transient species such as …

[PDF][PDF] Prion-like properties of pathological TDP-43 aggregates from diseased brains

T Nonaka, M Masuda-Suzukake, T Arai, Y Hasegawa… - Cell reports, 2013 - cell.com
TDP-43 is the major component protein of ubiquitin-positive inclusions in brains of patients
with frontotemporal lobar degeneration (FTLD-TDP) or amyotrophic lateral sclerosis (ALS) …

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

AC Elden, HJ Kim, MP Hart, AS Chen-Plotkin… - Nature, 2010 - nature.com
The causes of amyotrophic lateral sclerosis (ALS), a devastating human neurodegenerative
disease, are poorly understood, although the protein TDP-43 has been suggested to have a …

TDP-43 is intercellularly transmitted across axon terminals

MS Feiler, B Strobel, A Freischmidt, AM Helferich… - Journal of Cell …, 2015 - rupress.org
Transactive response DNA-binding protein 43 kD (TDP-43) is an aggregation-prone prion-
like domain-containing protein and component of pathological intracellular aggregates …

Atomic structures of TDP-43 LCD segments and insights into reversible or pathogenic aggregation

EL Guenther, Q Cao, H Trinh, J Lu… - Nature structural & …, 2018 - nature.com
The normally soluble TAR DNA-binding protein 43 (TDP-43) is found aggregated both in
reversible stress granules and in irreversible pathogenic amyloid. In TDP-43, the low …

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …