Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress

F Rojas, N Cortes, S Abarzua, A Dyrda… - Frontiers in cellular …, 2014 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal paralytic disorder caused by dysfunction and
degeneration of motor neurons. Multiple disease-causing mutations, including in the genes …

Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability

E Fritz, P Izaurieta, A Weiss, FR Mir… - Journal of …, 2013 - journals.physiology.org
Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by dysfunction
and degeneration of motoneurons starting in adulthood. Recent studies using cell or animal …

IFNγ triggers a LIGHT-dependent selective death of motoneurons contributing to the non-cell-autonomous effects of mutant SOD1

J Aebischer, P Cassina, B Otsmane… - Cell Death & …, 2011 - nature.com
Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease that primarily
affects motoneurons in the brain and spinal cord. Dominant mutations in superoxide …

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

M Nagai, DB Re, T Nagata, A Chalazonitis… - Nature …, 2007 - nature.com
Mutations in superoxide dismutase-1 (SOD1) cause a form of the fatal paralytic disorder
amyotrophic lateral sclerosis (ALS), presumably by a combination of cell-autonomous and …

Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis

P Díaz-Amarilla, S Olivera-Bravo… - Proceedings of the …, 2011 - National Acad Sciences
Motoneuron loss and reactive astrocytosis are pathological hallmarks of amyotrophic lateral
sclerosis (ALS), a paralytic neurodegenerative disease that can be triggered by mutations in …

A Drosophila Model for Amyotrophic Lateral Sclerosis Reveals Motor Neuron Damage by Human SOD1*♦

MR Watson, RD Lagow, K Xu, B Zhang… - Journal of Biological …, 2008 - ASBMB
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease that leads to loss of motor
function and early death. About 5% of cases are inherited, with the majority of identified …

SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments

PI Joyce, P Fratta, EMC Fisher… - Mammalian Genome, 2011 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease with no cure.
Breakthroughs in understanding ALS pathogenesis came with the discovery of dominant …

Wildtype motoneurons, ALS‐Linked SOD1 mutation and glutamate profoundly modify astrocyte metabolism and lactate shuttling

B Madji Hounoum, S Mavel, E Coque, F Patin… - Glia, 2017 - Wiley Online Library
The selective degeneration of motoneuron that typifies amyotrophic lateral sclerosis (ALS)
implicates non‐cell‐autonomous effects of astrocytes. However, mechanisms underlying …

Motoneuron death triggered by a specific pathway downstream of Fas: potentiation by ALS-linked SOD1 mutations

C Raoul, AG Estévez, H Nishimune, DW Cleveland… - Neuron, 2002 - cell.com
Death pathways restricted to specific neuronal classes could potentially allow for precise
control of developmental neuronal death and also underlie the selectivity of neuronal loss in …

Chronic activation in presymptomatic amyotrophic lateral sclerosis (ALS) mice of a feedback loop involving Fas, Daxx, and FasL

C Raoul, E Buhler, C Sadeghi… - Proceedings of the …, 2006 - National Acad Sciences
The reasons for the cellular specificity and slow progression of motoneuron diseases such
as ALS are still poorly understood. We previously described a motoneuron-specific cell …