[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 …

ALS: a disease of motor neurons and their nonneuronal neighbors

S Boillée, CV Velde, DW Cleveland - Neuron, 2006 - cell.com
Amyotrophic lateral sclerosis is a late-onset progressive neurodegenerative disease
affecting motor neurons. The etiology of most ALS cases remains unknown, but 2% of …

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and-independent mechanisms

LI Grad, JJ Yerbury, BJ Turner… - Proceedings of the …, 2014 - National Acad Sciences
Amyotrophic lateral sclerosis (ALS) is predominantly sporadic, but associated with heritable
genetic mutations in 5–10% of cases, including those in Cu/Zn superoxide dismutase …

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

DA Bosco, G Morfini, NM Karabacak, Y Song… - Nature …, 2010 - nature.com
Many mutations confer one or more toxic function (s) on copper/zinc superoxide dismutase 1
(SOD1) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis …

Update on the oxidative stress theory of aging: does oxidative stress play a role in aging or healthy aging?

AB Salmon, A Richardson, VI Pérez - Free Radical Biology and Medicine, 2010 - Elsevier
The oxidative stress theory of aging predicts that manipulations that alter oxidative
stress/damage will alter aging. The gold standard for determining whether aging is altered is …

[HTML][HTML] Liquid-liquid phase separation as a common organizing principle of intracellular space and biomembranes providing dynamic adaptive responses

SV Nesterov, NS Ilyinsky, VN Uversky - Biochimica et Biophysica Acta (BBA …, 2021 - Elsevier
This work is devoted to the phenomenon of liquid-liquid phase separation (LLPS), which has
come to be recognized as fundamental organizing principle of living cells. We distinguish …

[HTML][HTML] Sporadic and hereditary amyotrophic lateral sclerosis (ALS)

S Ajroud-Driss, T Siddique - … et Biophysica Acta (BBA)-Molecular Basis of …, 2015 - Elsevier
Genetic discoveries in ALS have a significant impact on deciphering molecular mechanisms
of motor neuron degeneration. The identification of SOD1 as the first genetic cause of ALS …

Lysozyme: a model protein for amyloid research

R Swaminathan, VK Ravi, S Kumar, MVS Kumar… - Advances in protein …, 2011 - Elsevier
Ever since lysozyme was discovered by Fleming in 1922, this protein has emerged as a
model for investigations on protein structure and function. Over the years, several high …

An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

MS Rotunno, DA Bosco - Frontiers in cellular neuroscience, 2013 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that targets motor
neurons, leading to paralysis and death within a few years of disease onset. While several …

RETRACTED: Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

JD Atkin, MA Farg, AK Walker, C McLean, D Tomas… - 2008 - Elsevier
The unfolded protein response (UPR) is induced at symptom onset and disease end stage
in rodent models of familial amyotrophic lateral sclerosis (ALS) that express superoxide …