Excitotoxicity in ALS: overstimulation, or overreaction?

AE King, A Woodhouse, MTK Kirkcaldie… - Experimental neurology, 2016 - Elsevier
Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disease that results
in motor dysfunction and death, generally from respiratory failure. 90% of ALS cases are …

Phase separation and pathologic transitions of RNP condensates in neurons: implications for amyotrophic lateral sclerosis, frontotemporal dementia and other …

A Naskar, A Nayak, MR Salaikumaran… - Frontiers in Molecular …, 2023 - frontiersin.org
Liquid–liquid phase separation results in the formation of dynamic biomolecular
condensates, also known as membrane-less organelles, that allow for the assembly of …

Distinct roles for motor neuron autophagy early and late in the SOD1G93A mouse model of ALS

ND Rudnick, CJ Griffey, P Guarnieri… - Proceedings of the …, 2017 - National Acad Sciences
Mutations in autophagy genes can cause familial and sporadic amyotrophic lateral sclerosis
(ALS). However, the role of autophagy in ALS pathogenesis is poorly understood, in part …

MicroRNA-206: a potential circulating biomarker candidate for amyotrophic lateral sclerosis

JM Toivonen, R Manzano, S Oliván, P Zaragoza… - PLoS …, 2014 - journals.plos.org
Amyotrophic lateral sclerosis (ALS) is a lethal motor neuron disease that progressively
debilitates neuronal cells that control voluntary muscle activity. Biomarkers are urgently …

Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis

F Leroy, B Lamotte d'Incamps, RD Imhoff-Manuel… - Elife, 2014 - elifesciences.org
In amyotrophic lateral sclerosis (ALS) the large motoneurons that innervate the fast-
contracting muscle fibers (F-type motoneurons) are vulnerable and degenerate in …

Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS

M Lalancette-Hebert, A Sharma… - Proceedings of the …, 2016 - National Acad Sciences
The molecular and cellular basis of selective motor neuron (MN) vulnerability in amyotrophic
lateral sclerosis (ALS) is not known. In genetically distinct mouse models of familial ALS …

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 …

Spinal V1 inhibitory interneuron clades differ in birthdate, projections to motoneurons, and heterogeneity

AE Worthy, JAT Anderson, AR Lane, LJ Gomez-Perez… - eLife, 2024 - elifesciences.org
Spinal cord interneurons play critical roles shaping motor output, but their precise identity
and connectivity remain unclear. Focusing on the V1 interneuron cardinal class we defined …

Spinal inhibitory neurons degenerate before motor neurons and excitatory neurons in a mouse model of ALS

R Montañana-Rosell, R Selvan… - Science …, 2024 - science.org
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive loss of somatic motor
neurons. A major focus has been directed to motor neuron intrinsic properties as a cause for …

Degeneration of proprioceptive sensory nerve endings in mice harboring amyotrophic lateral sclerosis–causing mutations

SK Vaughan, Z Kemp, T Hatzipetros… - Journal of …, 2015 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that primarily targets the
motor system. Although much is known about the effects of ALS on motor neurons and glial …