[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 …

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 …

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 …

In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants

E Luchinat, L Barbieri, JT Rubino, T Kozyreva… - Nature …, 2014‏ - nature.com
Mutations in the superoxide dismutase 1 (SOD1) gene are related to familial cases of
amyotrophic lateral sclerosis (fALS). Here we exploit in-cell NMR to characterize the protein …

Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity

L McAlary, JA Aquilina, JJ Yerbury - Frontiers in neuroscience, 2016‏ - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the rapid and progressive degeneration of upper and lower motor neurons in the spinal …

SOD1 aggregation and ALS: role of metallation states and disulfide status

Y Sheng, M Chattopadhyay… - Current topics in …, 2012‏ - benthamdirect.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the death of motor neurons. About 10% of ALS cases are inherited (familial), and a large …

Mass spectrometry imaging of SOD1 protein-metal complexes in SOD1G93A transgenic mice implicates demetalation with pathology

OJ Hale, TR Wells, RJ Mead, HJ Cooper - Nature Communications, 2024‏ - nature.com
Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motor neurons in the
central nervous system (CNS). Mutations in the metalloenzyme SOD1 are associated with …

[HTML][HTML] The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro

JJ Yerbury, D Gower, L Vanags, K Roberts… - Cell Stress and …, 2013‏ - Elsevier
Amyotrophic lateral sclerosis is a devastating neurodegenerative disease. The mechanism
that underlies amyotrophic lateral sclerosis (ALS) pathology remains unclear, but protein …

Redox regulation in amyotrophic lateral sclerosis

S Parakh, DM Spencer, MA Halloran… - Oxidative medicine …, 2013‏ - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the
death of upper and lower motor neurons. Due to a lack of effective treatment, it is imperative …

Characterization of the activity, aggregation, and toxicity of heterodimers of WT and ALS-associated mutant Sod1

AA Brasil, MDC de Carvalho, E Gerhardt… - Proceedings of the …, 2019‏ - pnas.org
Mutations in Cu/Zn superoxide dismutase (Sod1) have been reported in both familial and
sporadic amyotrophic lateral sclerosis (ALS). In this study, we investigated the behavior of …