ALS genetics: gains, losses, and implications for future therapies

G Kim, O Gautier, E Tassoni-Tsuchida, XR Ma… - Neuron, 2020 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the loss
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …

Chemically diverse small molecule fluorescent chemosensors for copper ion

G Sivaraman, M Iniya, T Anand, NG Kotla… - Coordination Chemistry …, 2018 - Elsevier
The development of sensitive chromogenic chemosensors for sensing Cu 2+ has been
receiving much attention in recent years because of their potential application in clinical …

Superoxide dismutases and superoxide reductases

Y Sheng, IA Abreu, DE Cabelli, MJ Maroney… - Chemical …, 2014 - ACS Publications
Superoxide, O2•−, is formed in all living organisms that come in contact with air, and,
depending upon its biological context, it may act as a signaling agent, a toxic species, or a …

Synthetic fluorescent probes for studying copper in biological systems

JA Cotruvo Jr, AT Aron, KM Ramos-Torres… - Chemical Society …, 2015 - pubs.rsc.org
The potent redox activity of copper is required for sustaining life. Mismanagement of its
cellular pools, however, can result in oxidative stress and damage connected to aging …

Bioinorganic chemistry of Alzheimer's disease

KP Kepp - Chemical reviews, 2012 - ACS Publications
Alzheimer's disease (AD) 1− 3 is the most common form of dementia (estimated∼ 50− 60%
of all cases), associated with loss of memory (in particular episodic memory), cognitive …

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 …

Copper homeostasis and neurodegenerative disorders (Alzheimer's, prion, and Parkinson's diseases and amyotrophic lateral sclerosis)

E Gaggelli, H Kozlowski, D Valensin… - Chemical …, 2006 - ACS Publications
Copper is too redox active to exist in an unbound form in the cell without causing oxidative
damage: an upper limit of 10-18 M for the free concentration of Cu (II) in unstressed cells has …

Superoxide dismutases—a review of the metal-associated mechanistic variations

IA Abreu, DE Cabelli - Biochimica et Biophysica Acta (BBA)-Proteins and …, 2010 - Elsevier
Superoxide dismutases are enzymes that function to catalytically convert superoxide radical
to oxygen and hydrogen peroxide. These enzymes carry out catalysis at near diffusion …

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 …

Non-cell-autonomous pathogenic mechanisms in amyotrophic lateral sclerosis

ACM Van Harten, H Phatnani, S Przedborski - Trends in neurosciences, 2021 - cell.com
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset paralytic disorder,
characterized mainly by a loss of motor neurons (MNs) in the CNS. Over the past decades …