Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

RJ Mead, N Shan, HJ Reiser, F Marshall… - Nature Reviews Drug …, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of
motor neurons. As with all major neurodegenerative disorders, development of disease …

[HTML][HTML] 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 …

Role of neuroinflammation in amyotrophic lateral sclerosis: cellular mechanisms and therapeutic implications

J Liu, F Wang - Frontiers in immunology, 2017 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects
upper motor neurons (MNs) comprising the corticospinal tract and lower MNs arising from …

The C9orf72 protein interacts with Rab1a and the ULK 1 complex to regulate initiation of autophagy

CP Webster, EF Smith, CS Bauer, A Moller… - The EMBO …, 2016 - embopress.org
A GGGGCC hexanucleotide repeat expansion in the C9orf72 gene is the most common
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9 ALS/FTD) …

Astrocytes in neurodegenerative disease

H Phatnani, T Maniatis - Cold Spring Harbor …, 2015 - cshperspectives.cshlp.org
Astrocytes contribute to the maintenance of the health and function of the central nervous
system (CNS). Thus, it is not surprising that these multifunctional cells have been implicated …

Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis

J Nijssen, LH Comley, E Hedlund - Acta neuropathologica, 2017 - Springer
In the fatal disease—amyotrophic lateral sclerosis (ALS)—upper (corticospinal) motor
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate …

Young glial progenitor cells competitively replace aged and diseased human glia in the adult chimeric mouse brain

R Vieira, JN Mariani, NPT Huynh… - Nature …, 2024 - nature.com
Competition among adult brain cells has not been extensively researched. To investigate
whether healthy glia can outcompete diseased human glia in the adult forebrain, we …

Meta-analysis of human and mouse ALS astrocytes reveals multi-omic signatures of inflammatory reactive states

OJ Ziff, BE Clarke, DM Taha, H Crerar… - Genome …, 2022 - genome.cshlp.org
Astrocytes contribute to motor neuron death in amyotrophic lateral sclerosis (ALS), but
whether they adopt deleterious features consistent with inflammatory reactive states remains …

Functional cortical neurons and astrocytes from human pluripotent stem cells in 3D culture

AM Paşca, SA Sloan, LE Clarke, Y Tian… - Nature …, 2015 - nature.com
The human cerebral cortex develops through an elaborate succession of cellular events
that, when disrupted, can lead to neuropsychiatric disease. The ability to reprogram somatic …

[HTML][HTML] Human stem cell models of neurodegeneration: From basic science of amyotrophic lateral sclerosis to clinical translation

E Giacomelli, BF Vahsen, EL Calder, Y Xu, J Scaber… - Cell Stem Cell, 2022 - cell.com
Neurodegenerative diseases are characterized by progressive cell loss leading to disruption
of the structure and function of the central nervous system. Amyotrophic lateral sclerosis …