[HTML][HTML] Phase separation and neurodegenerative diseases: a disturbance in the force

A Zbinden, M Pérez-Berlanga, P De Rossi… - Developmental cell, 2020 - cell.com
Protein aggregation is the main hallmark of neurodegenerative diseases. Many proteins
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …

The phenotypic variability of amyotrophic lateral sclerosis

B Swinnen, W Robberecht - Nature Reviews Neurology, 2014 - nature.com
Classic textbook neurology teaches that amyotrophic lateral sclerosis (ALS) is a
degenerative disease that selectively affects upper and lower motor neurons and is fatal 3–5 …

Extracellular vesicles in neurodegenerative disease—pathogenesis to biomarkers

AG Thompson, E Gray, SM Heman-Ackah… - Nature Reviews …, 2016 - nature.com
To develop effective disease-modifying therapies for neurodegenerative diseases, reliable
markers of diagnosis, disease activity and progression are a research priority. The fact that …

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

HX Deng, W Chen, ST Hong, KM Boycott, GH Gorrie… - Nature, 2011 - nature.com
Amyotrophic lateral sclerosis (ALS) is a paralytic and usually fatal disorder caused by motor-
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …

Molecular and cellular mechanisms affected in ALS

L Le Gall, E Anakor, O Connolly… - Journal of personalized …, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …

PROTOFIBRILS, PORES, FIBRILS, AND NEURODEGENERATION: Separating the Responsible Protein Aggregates from The Innocent Bystanders

B Caughey, PT Lansbury Jr - Annual review of neuroscience, 2003 - annualreviews.org
▪ Abstract Many neurodegenerative diseases, including Alzheimer's and Parkinson's and the
transmissible spongiform encephalopathies (prion diseases), are characterized at autopsy …

ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions

LI Bruijn, MW Becher, MK Lee, KL Anderson… - Neuron, 1997 - cell.com
High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and
G37R were previously shown to mediate disease in mice through an acquired toxic property …

Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1

LI Bruijn, MK Houseweart, S Kato, KL Anderson… - Science, 1998 - science.org
Analysis of transgenic mice expressing familial amyotrophic lateral sclerosis (ALS)–linked
mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron …

The structural biochemistry of the superoxide dismutases

JJP Perry, DS Shin, ED Getzoff, JA Tainer - Biochimica et Biophysica Acta …, 2010 - Elsevier
The discovery of superoxide dismutases (SODs), which convert superoxide radicals to
molecular oxygen and hydrogen peroxide, has been termed the most important discovery of …

Oxidative stress in ALS: key role in motor neuron injury and therapeutic target

SC Barber, PJ Shaw - Free Radical Biology and Medicine, 2010 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder
characterized by death of motor neurons leading to muscle wasting, paralysis, and death …