Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD

Y Tamaki, M Urushitani - International journal of molecular sciences, 2022 - mdpi.com
TAR DNA binding protein 43 (TDP-43) is a DNA/RNA binding protein involved in pivotal
cellular functions, especially in RNA metabolism. Hyperphosphorylated and ubiquitinated …

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 …

Prion-like propagation of protein misfolding and aggregation in amyotrophic lateral sclerosis

L McAlary, SS Plotkin, JJ Yerbury… - Frontiers in molecular …, 2019 - frontiersin.org
The discovery that prion protein can misfold into a pathological conformation that encodes
structural information capable of both propagation and inducing severe neuropathology has …

[HTML][HTML] Molecular mechanisms of phase separation and amyloidosis of ALS/FTD-linked FUS and TDP-43

J Song - Aging and Disease, 2024 - pmc.ncbi.nlm.nih.gov
FUS and TDP-43, two RNA-binding proteins from the heterogeneous nuclear
ribonucleoprotein family, have gained significant attention in the field of neurodegenerative …

Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS

K Weskamp, EM Tank, R Miguez… - The Journal of …, 2020 - Am Soc Clin Investig
Cortical hyperexcitability and mislocalization of the RNA-binding protein TDP43 are highly
conserved features in amyotrophic lateral sclerosis (ALS). Nevertheless, the relationship …

[HTML][HTML] Valency and binding affinity variations can regulate the multilayered organization of protein condensates with many components

I Sanchez-Burgos, JR Espinosa, JA Joseph… - Biomolecules, 2021 - mdpi.com
Biomolecular condensates, which assemble via the process of liquid–liquid phase
separation (LLPS), are multicomponent compartments found ubiquitously inside cells …

Potential therapeutic role of HDAC inhibitors in FUS-ALS

C Tejido, D Pakravan, LVD Bosch - Frontiers in Molecular …, 2021 - frontiersin.org
Mutations in the FUS gene cause amyotrophic lateral sclerosis (ALS-FUS). However, the
exact pathogenic mechanism of mutant fused in sarcoma (FUS) protein is not completely …

ATP is a cryptic binder of TDP-43 RRM domains to enhance stability and inhibit ALS/AD-associated fibrillation

M Dang, J Kang, L Lim, Y Li, L Wang, J Song - … and Biophysical Research …, 2020 - Elsevier
ATP is the universal energy currency for all cells but has cellular concentrations of 2–12 mM,
much higher than required for its classic functions. RNA-recognition motif (RRM) constitutes …

Sialotranscriptomics of the argasid tick Ornithodoros moubata along the trophogonic cycle

A Oleaga, B Soriano, C Llorens… - PLoS neglected …, 2021 - journals.plos.org
The argasid tick Ornithodoros moubata is the main vector of human relapsing fever (HRF)
and African swine fever (ASF) in Africa. Salivary proteins are part of the host-tick interface …

RNA-seq analysis and gene expression dynamics in the salivary glands of the argasid tick Ornithodoros erraticus along the trophogonic cycle

R Pérez-Sánchez, Á Carnero-Morán, B Soriano… - Parasites & …, 2021 - Springer
Abstract Background The argasid tick Ornithodoros erraticus is the main vector of tick-borne
human relapsing fever (TBRF) and African swine fever (ASF) in the Mediterranean Basin …