[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] Calcium permeable-AMPA receptors and excitotoxicity in neurological disorders

C Guo, YY Ma - Frontiers in neural circuits, 2021 - frontiersin.org
Excitotoxicity is one of the primary mechanisms of cell loss in a variety of diseases of the
central and peripheral nervous systems. Other than the previously established signaling …

Stress granules as crucibles of ALS pathogenesis

YR Li, OD King, J Shorter, AD Gitler - Journal of cell biology, 2013 - rupress.org
Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …

[HTML][HTML] TDP-43 proteinopathy and ALS: insights into disease mechanisms and therapeutic targets

EL Scotter, HJ Chen, CE Shaw - Neurotherapeutics, 2015 - Elsevier
Therapeutic options for patients with amyotrophic lateral sclerosis (ALS) are currently
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …

Updated TDP-43 in Alzheimer's disease staging scheme

KA Josephs, ME Murray, JL Whitwell… - Acta …, 2016 - Springer
In this study, we update the TDP-43 in Alzheimer's disease staging scheme by assessing the
topography of TDP-43 in 193 cases of Alzheimer's disease, in 14 different brain regions …

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

RC Hergesheimer, AA Chami, DR De Assis, P Vourc'h… - Brain, 2019 - academic.oup.com
Transactive response DNA-binding protein-43 (TDP-43) is an RNA/DNA binding protein that
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …

Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis

SJ Barmada, G Skibinski, E Korb, EJ Rao… - Journal of …, 2010 - Soc Neuroscience
Mutations in the gene encoding TDP-43—the major protein component of neuronal
aggregates characteristic of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar …

[HTML][HTML] Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS

Z Sun, Z Diaz, X Fang, MP Hart, A Chesi, J Shorter… - PLoS …, 2011 - journals.plos.org
TDP-43 and FUS are RNA-binding proteins that form cytoplasmic inclusions in some forms
of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) …

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

IF Wang, BS Guo, YC Liu, CC Wu… - Proceedings of the …, 2012 - National Acad Sciences
TDP-43 is a multifunctional DNA/RNA-binding protein that has been identified as the major
component of the cytoplasmic ubiquitin (+) inclusions (UBIs) in diseased cells of …

TAR DNA-binding protein 43 in neurodegenerative disease

AS Chen-Plotkin, VMY Lee… - Nature Reviews …, 2010 - nature.com
In 2006, TAR DNA-binding protein 43 (TDP-43), a highly conserved nuclear protein, was
identified as the major disease protein in amyotrophic lateral sclerosis (ALS) and in the most …