[HTML][HTML] 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] Roles of tau protein in health and disease

T Guo, W Noble, DP Hanger - Acta neuropathologica, 2017 - Springer
Tau is well established as a microtubule-associated protein in neurons. However, under
pathological conditions, aberrant assembly of tau into insoluble aggregates is accompanied …

[HTML][HTML] TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

CC Chou, YI Zhang, ME Umoh, SW Vaughan… - Nature …, 2018 - nature.com
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43)
is a common histopathological hallmark of the amyotrophic lateral sclerosis and …

[HTML][HTML] Poly (ADP-ribose) prevents pathological phase separation of TDP-43 by promoting liquid demixing and stress granule localization

L McGurk, E Gomes, L Guo, J Mojsilovic-Petrovic… - Molecular cell, 2018 - cell.com
In amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD), cytoplasmic
aggregates of hyperphosphorylated TDP-43 accumulate and colocalize with some stress …

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

LA Becker, B Huang, G Bieri, R Ma, DA Knowles… - Nature, 2017 - nature.com
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that
is characterized by motor neuron loss and that leads to paralysis and death 2–5 years after …

TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS

X Zuo, J Zhou, Y Li, K Wu, Z Chen, Z Luo… - Nature structural & …, 2021 - nature.com
Amyotrophic lateral sclerosis (ALS) was initially thought to be associated with oxidative
stress when it was first linked to mutant superoxide dismutase 1 (SOD1). The subsequent …

[HTML][HTML] TDP-43 pathology in Alzheimer's disease

A Meneses, S Koga, J O'Leary, DW Dickson… - Molecular …, 2021 - Springer
Transactive response DNA binding protein of 43 kDa (TDP-43) is an intranuclear protein
encoded by the TARDBP gene that is involved in RNA splicing, trafficking, stabilization, and …

[HTML][HTML] Tau protein interaction partners and their roles in Alzheimer's disease and other tauopathies

J Sinsky, K Pichlerova, J Hanes - International journal of molecular …, 2021 - mdpi.com
Tau protein plays a critical role in the assembly, stabilization, and modulation of
microtubules, which are important for the normal function of neurons and the brain. In …

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 …