Current insights in the molecular genetic pathogenesis of amyotrophic lateral sclerosis

W Zhou, R Xu - Frontiers in Neuroscience, 2023 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease
that leads to the massive loss of motor neurons in cerebrum, brain stem and spinal cord. It …

Valosin containing protein (VCP): a multistep regulator of autophagy

V Ferrari, R Cristofani, B Tedesco, V Crippa… - International Journal of …, 2022 - mdpi.com
Valosin containing protein (VCP) has emerged as a central protein in the regulation of the
protein quality control (PQC) system. VCP mutations are causative of multisystem …

Protein kinetics of superoxide dismutase‐1 in familial and sporadic amyotrophic lateral sclerosis

CV Ly, MD Ireland, WK Self, J Bollinger… - Annals of Clinical …, 2023 - Wiley Online Library
Objective Accumulation of misfolded superoxide dismutase‐1 (SOD1) is a pathological
hallmark of SOD1‐related amyotrophic lateral sclerosis (ALS) and is observed in sporadic …

Meiotic resetting of the cellular Sod1 pool is driven by protein aggregation, degradation, and transient LUTI-mediated repression

HM Vander Wende, M Gopi, M Onyundo… - Journal of Cell …, 2023 - rupress.org
Gametogenesis requires packaging of the cellular components needed for the next
generation. In budding yeast, this process includes degradation of many mitotically stable …

Variability in SOD1-associated amyotrophic lateral sclerosis: geographic patterns, clinical heterogeneity, molecular alterations, and therapeutic implications

M Huang, YU Liu, X Yao, D Qin, H Su - Translational Neurodegeneration, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
progressive loss of motor neurons, resulting in global health burden and limited post …

Amyloidogenic regions in beta-strands II and III modulate the aggregation and toxicity of SOD1 in living cells

L McAlary, JR Nan, C Shyu, M Sher… - Open …, 2024 - royalsocietypublishing.org
Mutations in the protein superoxide dismutase-1 (SOD1) promote its misfolding and
aggregation, ultimately causing familial forms of the debilitating neurodegenerative disease …

Meiotic resetting of the cellular Sod1 pool is driven by protein aggregation, degradation, and transient LUTI-mediated repression

HMV Wende, M Gopi, M Onyundo, C Medrano… - bioRxiv, 2022 - biorxiv.org
Gametogenesis requires packaging of the cellular components needed for the next
generation. In budding yeast, this process includes degradation of many mitotically stable …

Creating and characterising physiological mouse models of SOD1-and C9orf72-ALS

D Thompson - 2024 - ora.ox.ac.uk
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
characterised by the loss of motor neurons. Mutations in the superoxide dismutase 1 (SOD1) …

[图书][B] Characterization of the meiotic regulation of Superoxide dismutase 1 in Saccharomyces cerevisiae

HM Vander Wende - 2022 - search.proquest.com
Meiosis is the conserved cell division process used by sexually reproducing organisms to
produce gametes, which are the cells that give rise to the next generation. In humans …