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 …

Physicochemical properties of cells and their effects on intrinsically disordered proteins (IDPs)

FX Theillet, A Binolfi, T Frembgen-Kesner… - Chemical …, 2014 - ACS Publications
It has long been axiomatic that a protein's structure determines its function. Intrinsically
disordered proteins (IDPs) and disordered protein regions (IDRs) defy this structure …

[HTML][HTML] Passive immunotherapies targeting Aβ and tau in Alzheimer's disease

SS Plotkin, NR Cashman - Neurobiology of Disease, 2020 - Elsevier
Amyloid-β (Aβ) and tau proteins currently represent the two most promising targets to treat
Alzheimer's disease. The most extensively developed method to treat the pathologic forms of …

Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

IR Mackenzie, T Arzberger, E Kremmer, D Troost… - Acta …, 2013 - Springer
Hexanucleotide repeat expansion in C9ORF72 is the most common genetic cause of
frontotemporal dementia and motor neuron disease. Recently, unconventional non-ATG …

Mechanisms of protein-folding diseases at a glance

JS Valastyan, S Lindquist - Disease models & mechanisms, 2014 - journals.biologists.com
For a protein to function appropriately, it must first achieve its proper conformation and
location within the crowded environment inside the cell. Multiple chaperone systems are …

Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers

IRA Mackenzie, P Frick, FA Grässer, TF Gendron… - Acta …, 2015 - Springer
Hexanucleotide repeat expansion in C9ORF72 is the most common genetic cause of
frontotemporal dementia and motor neuron disease. One consequence of the mutation is the …

[HTML][HTML] The role of amyloid oligomers in neurodegenerative pathologies

C Wells, S Brennan, M Keon, L Ooi - International Journal of Biological …, 2021 - Elsevier
Many neurodegenerative diseases are rooted in the activities of amyloid-like proteins which
possess conformations that spread to healthy proteins. These include Alzheimer's disease …

Insulin-degrading enzyme in the fight against Alzheimer's disease

IV Kurochkin, E Guarnera, IN Berezovsky - Trends in pharmacological …, 2018 - cell.com
After decades of research and clinical trials there is still no cure for Alzheimer's disease
(AD). While impaired clearance of amyloid beta (Aβ) peptides is considered as one of the …

Inhibition of amyloid formation

T Härd, C Lendel - Journal of molecular biology, 2012 - Elsevier
Amyloid is aggregated protein in the form of insoluble fibrils. Amyloid deposition in human
tissue—amyloidosis—is associated with a number of diseases including all common …

Modelling neurodegeneration in Saccharomyces cerevisiae: why cook with baker's yeast?

V Khurana, S Lindquist - Nature Reviews Neuroscience, 2010 - nature.com
In ageing populations, neurodegenerative diseases increase in prevalence, exacting an
enormous toll on individuals and their communities. Multiple complementary experimental …