Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …

Specialized subpopulations of deep-layer pyramidal neurons in the neocortex: bridging cellular properties to functional consequences

A Baker, B Kalmbach, M Morishima, J Kim… - Journal of …, 2018 - jneurosci.org
Neocortical pyramidal neurons with somata in layers 5 and 6 are among the most visually
striking and enigmatic neurons in the brain. These deep-layer pyramidal neurons (DLPNs) …

Motor learning promotes remyelination via new and surviving oligodendrocytes

CM Bacmeister, HJ Barr, CR McClain… - Nature …, 2020 - nature.com
Oligodendrocyte loss in neurological disease leaves axons vulnerable to damage and
degeneration, and activity-dependent myelination may represent an endogenous …

Regulation of cortical hyperexcitability in amyotrophic lateral sclerosis: focusing on glial mechanisms

M **e, PN Pallegar, S Parusel, AT Nguyen… - Molecular …, 2023 - Springer
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder
characterized by the loss of both upper and lower motor neurons, resulting in muscle …

How do we get from hyperexcitability to excitotoxicity in amyotrophic lateral sclerosis?

GL Odierna, S Vucic, M Dyer, T Dickson, A Woodhouse… - Brain, 2024 - academic.oup.com
Amyotrophic lateral sclerosis is a devastating neurodegenerative disease that, at present,
has no effective cure. Evidence of increased circulating glutamate and hyperexcitability of …

Revisiting glutamate excitotoxicity in amyotrophic lateral sclerosis and age-related neurodegeneration

FJ Arnold, AF Putka, U Raychaudhuri, S Hsu… - International Journal of …, 2024 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder. While there
are five FDA-approved drugs for treating this disease, each has only modest benefits. To …

Synaptic dysfunction and plasticity in amyotrophic lateral sclerosis

R Gulino - International Journal of Molecular Sciences, 2023 - mdpi.com
Recent evidence has supported the hypothesis that amyotrophic lateral sclerosis (ALS) is a
multi-step disease, as the onset of symptoms occurs after sequential exposure to a defined …

[HTML][HTML] Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons

P Harley, C Kerins, A Gatt, G Neves, F Riccio… - Cell Reports, 2023 - cell.com
Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We
sought to investigate how functional changes to the axon initial segment (AIS), the site of …

Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects

J Scekic-Zahirovic, I Sanjuan-Ruiz, V Kan… - Nature …, 2021 - nature.com
Gene mutations causing cytoplasmic mislocalization of the RNA-binding protein FUS lead to
severe forms of amyotrophic lateral sclerosis (ALS). Cytoplasmic accumulation of FUS is …

[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 …