Oxidative stress in neurodegenerative diseases

E Niedzielska, I Smaga, M Gawlik, A Moniczewski… - Molecular …, 2016 - Springer
The pathophysiologies of neurodegenerative diseases, including amyotrophic lateral
sclerosis (ALS), Parkinson's disease (PD), and Alzheimer's disease (AD), are far from being …

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 …

CCT2 is an aggrephagy receptor for clearance of solid protein aggregates

X Ma, C Lu, Y Chen, S Li, N Ma, X Tao, Y Li, J Wang… - Cell, 2022 - cell.com
Protein aggregation is a hallmark of multiple human pathologies. Autophagy selectively
degrades protein aggregates via aggrephagy. How selectivity is achieved has been elusive …

Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies

A Ciechanover, YT Kwon - Experimental & molecular medicine, 2015 - nature.com
Mammalian cells remove misfolded proteins using various proteolytic systems, including the
ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and …

Protein aggregation in amyotrophic lateral sclerosis

AM Blokhuis, EJN Groen, M Koppers… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …

The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins

V Deora, JD Lee, EA Albornoz, L McAlary, CJ Jagaraj… - Glia, 2020 - Wiley Online Library
Microglial NLRP3 inflammasome activation is emerging as a key contributor to
neuroinflammation during neurodegeneration. Pathogenic protein aggregates such as β …

Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

H Ilieva, M Polymenidou, DW Cleveland - Journal of Cell Biology, 2009 - rupress.org
Selective degeneration and death of one or more classes of neurons is the defining feature
of human neurodegenerative disease. Although traditionally viewed as diseases mainly …

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 …

Genetics of amyotrophic lateral sclerosis

M Ghasemi, RH Brown - Cold Spring …, 2018 - perspectivesinmedicine.cshlp.org
Amyotrophic lateral sclerosis (ALS) is a devastating, uniformly lethal degenerative disorder
of motor neurons that overlaps clinically with frontotemporal dementia (FTD). Investigations …