Molecular mechanisms underlying TDP-43 pathology in cellular and animal models of ALS and FTLD

A Wood, Y Gurfinkel, N Polain, W Lamont… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative disorders that exist on a disease spectrum due to pathological, clinical …

Oxidative stress in amyotrophic lateral sclerosis: synergy of genetic and environmental factors

A Motataianu, G Serban, L Barcutean… - International Journal of …, 2022 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a grievous neurodegenerative disease whose survival
is limited to only a few years. In spite of intensive research to discover the underlying …

Gel‐like inclusions of C‐terminal fragments of TDP‐43 sequester stalled proteasomes in neurons

H Riemenschneider, Q Guo, J Bader, F Frottin… - EMBO …, 2022 - embopress.org
Aggregation of the multifunctional RNA‐binding protein TDP‐43 defines large subgroups of
amyotrophic lateral sclerosis and frontotemporal dementia and correlates with …

Metamorphism in TDP-43 prion-like domain determines chaperone recognition

J Carrasco, R Antón, A Valbuena… - Nature …, 2023 - nature.com
The RNA binding protein TDP-43 forms cytoplasmic inclusions via its C-terminal prion-like
domain in several neurodegenerative diseases. Aberrant TDP-43 aggregation arises upon …

Stress induced TDP-43 mobility loss independent of stress granules

L Streit, T Kuhn, T Vomhof, V Bopp, AC Ludolph… - Nature …, 2022 - nature.com
Abstract TAR DNA binding protein 43 (TDP-43) is closely related to the pathogenesis of
amyotrophic lateral sclerosis (ALS) and translocates to stress granules (SGs). The role of …

Stress granule formation helps to mitigate neurodegeneration

MR Glineburg, E Yildirim, N Gomez… - Nucleic Acids …, 2024 - academic.oup.com
Cellular stress pathways that inhibit translation initiation lead to transient formation of
cytoplasmic RNA/protein complexes known as stress granules. Many of the proteins found …

Stress granules and neurodegenerative disorders: a scoping review

MR Asadi, M Sadat Moslehian, H Sabaie… - Frontiers in aging …, 2021 - frontiersin.org
Cytoplasmic ribonucleoproteins called stress granules (SGs) are considered as one of the
main cellular solutions against stress. Their temporary presence ends with stress relief. Any …

The many hats of transmembrane emp24 domain protein TMED9 in secretory pathway homeostasis

BS Roberts, P Satpute-Krishnan - Frontiers in Cell and …, 2023 - frontiersin.org
The secretory pathway is an intracellular highway for the vesicular transport of newly
synthesized proteins that spans the endoplasmic reticulum (ER), Golgi, lysosomes and the …

Atomistic insights into A315E mutation-enhanced pathogenicity of TDP-43 core fibrils

F Li, Y Chen, X Liu, Y Tang, X Dong… - ACS Chemical …, 2022 - ACS Publications
The aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) into fibrillary deposits is
implicated in amyotrophic lateral sclerosis (ALS), and some hereditary mutations localized in …

Data‐independent acquisition proteomics of cerebrospinal fluid implicates endoplasmic reticulum and inflammatory mechanisms in amyotrophic lateral sclerosis

ER Dellar, I Vendrell, K Talbot… - Journal of …, 2024 - Wiley Online Library
While unbiased proteomics of human cerebrospinal fluid (CSF) has been used successfully
to identify biomarkers of amyotrophic lateral sclerosis (ALS), high‐abundance proteins mask …