Molecular Mechanisms of Disease in ALS & FTD

Mutations in RNA-binding proteins, including TARDBP, have been found in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This feed focuses on molecular mechanisms that underlie ALS and FTD.

April 24, 2020
Open Access

Tissue-selective regulation of protein homeostasis and unfolded protein response signalling in sporadic ALS

Journal of Cellular and Molecular Medicine
Luigi MontibellerJacqueline de Belleroche
March 24, 2020
Review

Protein transmission in neurodegenerative disease

Nature Reviews. Neurology
Chao PengVirginia M-Y Lee
June 10, 2020
Review
Open Access

Cell-Clearing Systems Bridging Repeat Expansion Proteotoxicity and Neuromuscular Junction Alterations in ALS and SBMA

International Journal of Molecular Sciences
Fiona LimanaqiFrancesco Fornai
May 1, 2020
Review

Multiple MuSK signaling pathways and the aging neuromuscular junction

Neuroscience Letters
Lauren A Fish, Justin R Fallon
March 19, 2020
Open Access

Extensive splicing changes in an ALS/FTD transgenic mouse model overexpressing cytoplasmic fused in sarcoma

Scientific Reports
Daisuke ItoEiji Inoue
April 29, 2020

Cytoplasmic localization of amyotrophic lateral sclerosis (ALS)-related TDP-43 proteins modulates stress-granule formation

The European Journal of Neuroscience
Corinne Besnard-Guérin
August 4, 2020

Ablation of C9orf72 together with excitotoxicity induces ALS in rats

The FEBS Journal
Wei DongLianfeng Zhang
June 13, 2020
Review
Open Access

Potential Preventive Strategies for Amyotrophic Lateral Sclerosis

Frontiers in Neuroscience
B KuraszkiewiczM de Carvalho
June 20, 2020

Frontotemporal dementia: a conference report from the 2nd FinFTD Symposium

Neurodegenerative Disease Management
Dorit HoffmannAnnakaisa Haapasalo
March 20, 2020
Open Access

Friction-Limited Folding of Disulfide-Reduced Monomeric SOD1

Biophysical Journal
Noah R CohenC R Matthews
July 22, 2020

The protein-surfactant stoichiometry governs the conformational switching and amyloid nucleation kinetics of tau K18

European Biophysics Journal : EBJ
Jaspreet KaurMily Bhattacharya
June 11, 2020
Review
Open Access

Gut-Brain Axis and Neurodegeneration: State-of-the-Art of Meta-Omics Sciences for Microbiota Characterization

International Journal of Molecular Sciences
Bruno TiloccaViviana Greco
May 9, 2020

Poly (ADP-ribose) (PAR)-dependent cell death in neurodegenerative diseases

International Review of Cell and Molecular Biology
Hyejin ParkValina L Dawson
June 5, 2020

Common mutations of interest in the diagnosis of amyotrophic lateral sclerosis: how common are common mutations in ALS genes?

Expert Review of Molecular Diagnostics
Benedetta Perrone, Francesca Luisa Conforti
May 8, 2020
Open Access

Functional characterization of a FUS mutant zebrafish line as a novel genetic model for ALS

Neurobiology of Disease
Annis-Rayan BourefisEdor Kabashi
May 23, 2020
Review
Open Access

Genetic architecture of common non-Alzheimer's disease dementias

Neurobiology of Disease
Rita GuerreiroJose Bras
March 11, 2020
Review
Open Access

Programmed axon degeneration: from mouse to mechanism to medicine

Nature Reviews. Neuroscience
Michael P Coleman, Ahmet Höke

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