FUS and TDP-43 phases in health and disease

B Portz, BL Lee, J Shorter - Trends in biochemical sciences, 2021 - cell.com
The distinct prion-like domains (PrLDs) of FUS and TDP-43, modulate phase transitions that
result in condensates with a range of material states. These assemblies are implicated in …

Disrupting pathologic phase transitions in neurodegeneration

BT Hurtle, L Xie, CJ Donnelly - The Journal of clinical …, 2023 - Am Soc Clin Investig
Solid-like protein deposits found in aged and diseased human brains have revealed a
relationship between insoluble protein accumulations and the resulting deficits in neurologic …

[HTML][HTML] Emerging therapies and novel targets for TDP-43 proteinopathy in ALS/FTD

LR Hayes, P Kalab - Neurotherapeutics, 2022 - Elsevier
Nuclear clearance and cytoplasmic mislocalization of the essential RNA binding protein,
TDP-43, is a pathologic hallmark of amyotrophic lateral sclerosis, frontotemporal dementia …

New approaches to target RNA binding proteins

AR Julio, KM Backus - Current opinion in chemical biology, 2021 - Elsevier
RNA binding proteins (RBPs) are a large and diverse class of proteins that regulate all
aspects of RNA biology. As RBP dysregulation has been implicated in a number of human …

Animal models of neurodegenerative disease: Recent advances in fly highlight innovative approaches to drug discovery

JA Tello, HE Williams, RM Eppler… - Frontiers in molecular …, 2022 - frontiersin.org
Neurodegenerative diseases represent a formidable challenge to global health. As
advances in other areas of medicine grant healthy living into later decades of life, aging …

[HTML][HTML] TDP-43 as A Therapeutic Target in Neurodegenerative Diseases; Focusing on Motor Neuron Disease and Frontotemporal Dementia

A Babazadeh, SL Rayner, A Lee, RS Chung - Ageing Research Reviews, 2023 - Elsevier
A common feature of adult-onset neurodegenerative diseases is the presence of
characteristic pathological accumulations of specific proteins. These pathological protein …

Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology

L François-Moutal, DD Scott, AJ Ambrose, CJ Zerio… - Scientific reports, 2022 - nature.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with no cure or
effective treatment in which TAR DNA Binding Protein of 43 kDa (TDP-43) abnormally …

Reviewing the potential links between viral infections and TDP-43 proteinopathies

Z Rahic, E Buratti, S Cappelli - International Journal of Molecular …, 2023 - mdpi.com
Transactive response DNA binding protein 43 kDa (TDP-43) was discovered in 2001 as a
cellular factor capable to inhibit HIV-1 gene expression. Successively, it was brought to new …

[HTML][HTML] A novel Drosophila-based drug repurposing platform identified fingolimod as a potential therapeutic for TDP-43 proteinopathy

LL Piccolo, T Umegawachi, R Yeewa, S Potikanond… - …, 2023 - Elsevier
Pathogenic changes to TAR DNA-binding protein 43 (TDP-43) leading to alteration of its
homeostasis are a common feature shared by several progressive neurodegenerative …

Direct targeting of TDP-43, from small molecules to biologics: the therapeutic landscape

L Francois-Moutal, DD Scott, M Khanna - RSC Chemical Biology, 2021 - pubs.rsc.org
Tar DNA binding (TDP)-43 proteinopathy, typically described as cytoplasmic accumulation
of highly modified and misfolded TDP-43 molecules, is characteristic of several …