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 …

[HTML][HTML] SOD1, more than just an antioxidant

ECA Eleutherio, RSS Magalhães… - Archives of Biochemistry …, 2021 - Elsevier
During cellular respiration, radicals, such as superoxide, are produced, and in a large
concentration, they may cause cell damage. To combat this threat, the cell employs the …

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

JS Valentine, PA Doucette, S Zittin Potter - Annu. Rev. Biochem., 2005 - annualreviews.org
▪ Abstract Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant
copper-and zinc-containing protein that is present in the cytosol, nucleus, peroxisomes, and …

Mutant SOD1 mediated pathogenesis of amyotrophic lateral sclerosis

SJ Kaur, SR McKeown, S Rashid - Gene, 2016 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neural disorder that causes death of the motor
neurons in the brain and spinal cord; this affects the voluntary muscles and gradually leads …

[HTML][HTML] Activation of superoxide dismutases: putting the metal to the pedal

VC Culotta, M Yang, TV O'Halloran - Biochimica et Biophysica Acta (BBA) …, 2006 - Elsevier
Superoxide dismutases (SOD) are important anti-oxidant enzymes that guard against
superoxide toxicity. Various SOD enzymes have been characterized that employ either a …

Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic

BG Trist, JB Hilton, DJ Hare, PJ Crouch… - Angewandte Chemie …, 2021 - Wiley Online Library
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …

SOD1 in ALS: taking stock in pathogenic mechanisms and the role of glial and muscle cells

C Peggion, V Scalcon, ML Massimino, K Nies… - Antioxidants, 2022 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
the loss of motor neurons in the brain and spinal cord. While the exact causes of ALS are still …

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 …

The transition metals copper and iron in neurodegenerative diseases

S Rivera-Mancía, I Pérez-Neri, C Ríos… - Chemico-biological …, 2010 - Elsevier
Neurodegenerative diseases constitute a worldwide health problem. Metals like iron and
copper are essential for life, but they are also involved in several neurodegenerative …

Structural properties and interaction partners of familial ALS-associated SOD1 mutants

J Huai, Z Zhang - Frontiers in neurology, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron degenerative disease
in adults and has also been proven to be a type of conformational disease associated with …