Going the extra (synaptic) mile: excitotoxicity as the road toward neurodegenerative diseases

A Armada-Moreira, JI Gomes, CC Pina… - Frontiers in cellular …, 2020 - frontiersin.org
Excitotoxicity is a phenomenon that describes the toxic actions of excitatory
neurotransmitters, primarily glutamate, where the exacerbated or prolonged activation of …

Modeling ALS with motor neurons derived from human induced pluripotent stem cells

S Sances, LI Bruijn, S Chandran, K Eggan, R Ho… - Nature …, 2016 - nature.com
Directing the differentiation of induced pluripotent stem cells into motor neurons has allowed
investigators to develop new models of amyotrophic lateral sclerosis (ALS). However …

The role of altered BDNF/TrkB signaling in amyotrophic lateral sclerosis

J Pradhan, PG Noakes, MC Bellingham - Frontiers in cellular …, 2019 - frontiersin.org
Brain derived neurotrophic factor (BDNF) is well recognized for its neuroprotective functions,
via activation of its high affinity receptor, tropomysin related kinase B (TrkB). In addition …

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 …

Glial contribution to excitatory and inhibitory synapse loss in neurodegeneration

CM Henstridge, M Tzioras… - Frontiers in cellular …, 2019 - frontiersin.org
Synapse loss is an early feature shared by many neurodegenerative diseases, and it
represents the major correlate of cognitive impairment. Recent studies reveal that microglia …

ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

H An, L Skelt, A Notaro, JR Highley, AH Fox… - Acta neuropathologica …, 2019 - Springer
Mutations in the FUS gene cause amyotrophic lateral sclerosis (ALS-FUS). Mutant FUS is
known to confer cytoplasmic gain of function but its effects in the nucleus are less …

The role of DNA damage in neural plasticity in physiology and neurodegeneration

A Konopka, JD Atkin - Frontiers in Cellular Neuroscience, 2022 - frontiersin.org
Damage to DNA is generally considered to be a harmful process associated with aging and
aging-related disorders such as neurodegenerative diseases that involve the selective …

Amyotrophic lateral sclerosis: a long preclinical period?

A Eisen, M Kiernan, H Mitsumoto… - Journal of Neurology …, 2014 - jnnp.bmj.com
The onset of amyotrophic lateral sclerosis (ALS) is conventionally considered as
commencing with the recognition of clinical symptoms. We propose that, in common with …

Nuclear poly (ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

L McGurk, J Mojsilovic-Petrovic, VM Van Deerlin… - Acta neuropathologica …, 2018 - Springer
Amyotrophic lateral sclerosis (ALS) is a devastating and fatal motor neuron disease.
Diagnosis typically occurs in the fifth decade of life and the disease progresses rapidly …

[HTML][HTML] Modeling cell-autonomous motor neuron phenotypes in ALS using iPSCs

J Hawrot, S Imhof, BJ Wainger - Neurobiology of disease, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) is an aggressive and uniformly fatal degenerative
disease of the motor nervous system. In order to understand underlying disease …