Non-neuronal cells in amyotrophic lateral sclerosis—from pathogenesis to biomarkers

BF Vahsen, E Gray, AG Thompson, O Ansorge… - Nature Reviews …, 2021 - nature.com
The prevailing motor neuron-centric view of amyotrophic lateral sclerosis (ALS)
pathogenesis could be an important factor in the failure to identify disease-modifying therapy …

Human iPSC-derived neural models for studying Alzheimer's disease: from neural stem cells to cerebral organoids

M Barak, V Fedorova, V Pospisilova, J Raska… - Stem Cell Reviews and …, 2022 - Springer
During the past two decades, induced pluripotent stem cells (iPSCs) have been widely used
to study mechanisms of human neural development, disease modeling, and drug discovery …

Differentiation and maturation of oligodendrocytes in human three-dimensional neural cultures

RM Marton, Y Miura, SA Sloan, Q Li, O Revah… - Nature …, 2019 - nature.com
Investigating human oligodendrogenesis and the interaction of oligodendrocytes with
neurons and astrocytes would accelerate our understanding of the mechanisms underlying …

C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity

BT Selvaraj, MR Livesey, C Zhao, JM Gregory… - Nature …, 2018 - nature.com
Mutations in C9ORF72 are the most common cause of familial amyotrophic lateral sclerosis
(ALS). Here, through a combination of RNA-Seq and electrophysiological studies on …

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

L Ferraiuolo, K Meyer, TW Sherwood… - Proceedings of the …, 2016 - National Acad Sciences
Oligodendrocytes have recently been implicated in the pathophysiology of amyotrophic
lateral sclerosis (ALS). Here we show that, in vitro, mutant superoxide dismutase 1 (SOD1) …

Establishment of an a priori protocol for the implementation and interpretation of an in‐vitro testing battery for the assessment of developmental neurotoxicity

S Masjosthusmann, J Blum, K Bartmann… - EFSA Supporting …, 2020 - Wiley Online Library
In this project we set up a human cell‐based DNTin vitro testing strategy that is based on test
methods with high readiness and data generated therefrom. The methods underwent afit‐for …

CRISPR-edited human ES-derived oligodendrocyte progenitor cells improve remyelination in rodents

LJ Wagstaff, N Bestard-Cuche, M Kaczmarek… - Nature …, 2024 - nature.com
Abstract In Multiple Sclerosis (MS), inflammatory demyelinated lesions in the brain and
spinal cord lead to neurodegeneration and progressive disability. Remyelination can restore …

[HTML][HTML] iPSC-derived myelinoids to study myelin biology of humans

OG James, BT Selvaraj, D Magnani, K Burr… - Developmental Cell, 2021 - cell.com
Myelination is essential for central nervous system (CNS) formation, health, and function.
Emerging evidence of oligodendrocyte heterogeneity in health and disease and divergent …

Proteostatic imbalance and protein spreading in amyotrophic lateral sclerosis

ME Cicardi, L Marrone, M Azzouz, D Trotti - The EMBO journal, 2021 - embopress.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder whose exact
causative mechanisms are still under intense investigation. Several lines of evidence …

In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers

M DeJesus-Hernandez, NCA Finch, X Wang… - Acta …, 2017 - Springer
A growing body of evidence suggests that a loss of chromosome 9 open reading frame 72
(C9ORF72) expression, formation of dipeptide-repeat proteins, and generation of RNA foci …