Small-molecule modulation of TDP-43 recruitment to stress granules prevents persistent TDP-43 accumulation in ALS/FTD

MY Fang, S Markmiller, AQ Vu, A Javaherian… - Neuron, 2019 - cell.com
Stress granules (SGs) form during cellular stress and are implicated in neurodegenerative
diseases such as amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). To …

Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?

A Aulas, C Vande Velde - Frontiers in cellular neuroscience, 2015 - frontiersin.org
Stress granules (SGs) are RNA-containing cytoplasmic foci formed in response to stress
exposure. Since their discovery in 1999, over 120 proteins have been described to be …

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

Y Khalfallah, R Kuta, C Grasmuck, A Prat… - Scientific reports, 2018 - nature.com
Stress granules (SGs) are cytoplasmic foci that form in response to various external stimuli
and are essential to cell survival following stress. SGs are studied in several diseases …

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

W Guo, Y Chen, X Zhou, A Kar, P Ray, X Chen… - Nature structural & …, 2011 - nature.com
Mutations in TARDBP, encoding TAR DNA-binding protein-43 (TDP-43), are associated with
TDP-43 proteinopathies, including amyotrophic lateral sclerosis (ALS) and frontotemporal …

C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress

J Meyerowitz, SJ Parker, LJ Vella, DCH Ng… - Molecular …, 2011 - Springer
Background TDP-43 proteinopathies are characterized by loss of nuclear TDP-43
expression and formation of C-terminal TDP-43 fragmentation and accumulation in the …

ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation

AK Walker, KY Soo, V Sundaramoorthy, S Parakh… - PloS one, 2013 - journals.plos.org
In amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration, TAR DNA
binding protein 43 (TDP-43) accumulates in the cytoplasm of affected neurons and glia …

[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 …

Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP

A Aulas, S Stabile, C Vande Velde - Molecular neurodegeneration, 2012 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the selective loss of upper and lower motor neurons, a cell type that is intrinsically more …

Aggregation of the nucleic acid–binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation

Y Chen, TJ Cohen - Journal of biological chemistry, 2019 - ASBMB
TAR DNA-binding protein 43 (TDP-43) is a nucleic acid–binding protein, and its aggregation
represents the defining pathology in amyotrophic lateral sclerosis (ALS) and related …

[HTML][HTML] Chronic stress induces formation of stress granules and pathological TDP-43 aggregates in human ALS fibroblasts and iPSC-motoneurons

A Ratti, V Gumina, P Lenzi, P Bossolasco… - Neurobiology of …, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal
neurodegenerative diseases characterized by the presence of neuropathological …