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 …

The role of heparan sulfates in protein aggregation and their potential impact on neurodegeneration

A Maïza, S Chantepie, C Vera, A Fifre, MB Huynh… - FEBS …, 2018 - Wiley Online Library
Neurodegenerative disorders, such as Alzheimer's, Parkinson's, and prion diseases, are
directly linked to the formation and accumulation of protein aggregates in the brain. These …

Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS)

S Sangwan, A Zhao, KL Adams… - Proceedings of the …, 2017 - National Acad Sciences
Fibrils and oligomers are the aggregated protein agents of neuronal dysfunction in ALS
diseases. Whereas we now know much about fibril architecture, atomic structures of disease …

Implications of PI3K/AKT/PTEN signaling on superoxide dismutases expression and in the pathogenesis of Alzheimer's disease

S Matsuda, Y Nakagawa, A Tsuji, Y Kitagishi… - Diseases, 2018 - mdpi.com
Alzheimer's disease is a neurodegenerative sickness, where the speed of personal disease
progression differs prominently due to genetic and environmental factors such as life style …

Glycosaminoglycans in neurodegenerative diseases

W Jin, F Zhang, RJ Linhardt - The Role of Glycosylation in Health and …, 2021 - Springer
Glycosaminoglycans (GAGs) are linear polysaccharides that consist of alternating
disaccharides sequences of uronic acids and/or galactose hexamino sugars most of which …

Direct Observation of β-Barrel Intermediates in the Self-Assembly of Toxic SOD128–38 and Absence in Nontoxic Glycine Mutants

Y Sun, J Huang, X Duan, F Ding - Journal of chemical information …, 2021 - ACS Publications
Soluble low-molecular-weight oligomers formed during the early stage of amyloid
aggregation are considered the major toxic species in amyloidosis. The structure–function …

Premature termination codons in SOD1 causing Amyotrophic Lateral Sclerosis are predicted to escape the nonsense-mediated mRNA decay

C Guissart, K Mouzat, J Kantar, B Louveau, P Vilquin… - Scientific Reports, 2020 - nature.com
Amyotrophic lateral sclerosis (ALS) is the most common and severe adult-onset motoneuron
disease and has currently no effective therapy. Approximately 20% of familial ALS cases are …

Atomic structures of corkscrew‐forming segments of SOD1 reveal varied oligomer conformations

S Sangwan, MR Sawaya, KA Murray… - Protein …, 2018 - Wiley Online Library
The aggregation cascade of disease‐related amyloidogenic proteins, terminating in
insoluble amyloid fibrils, involves intermediate oligomeric states. The structural and …

Incorporating upper motor neuron health in ALS drug discovery

I Dervishi, PH Ozdinler - Drug discovery today, 2018 - Elsevier
Highlights•Inclusion of upper motor neuron health in preclinical screening is important.•Main
ALS mouse models investigating the role of CSMN are discussed.•Reporter lines of CSMN …

SOD1 in amyotrophic lateral sclerosis development–in silico analysis and molecular dynamics of A4F and A4V variants

ANR Da Silva, GRC Pereira… - Journal of Cellular …, 2019 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that is characterized by
the selective loss of motor neurons. Approximately 5% to 10% of patients with ALS have a …