Motor neuron susceptibility in ALS/FTD

AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

Nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives

T Bonifacino, RA Zerbo, M Balbi, C Torazza… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell
autonomous neurodegenerative disease characterized by upper and lower motor neuron …

Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology

AL Nana, M Sidhu, SE Gaus, JHL Hwang, L Li… - Acta …, 2019 - Springer
Abstract TAR DNA-binding protein 43 (TDP-43) aggregation is the most common
pathological hallmark in frontotemporal dementia (FTD) and characterizes nearly all patients …

Eukaryotic cell size regulation and its implications for cellular function and dysfunction

Y Chadha, A Khurana… - Physiological …, 2024 - journals.physiology.org
Depending on cell type, environmental inputs, and disease, the cells in the human body can
have widely different sizes. In recent years, it has become clear that cell size is a major …

Synaptic proteomics reveal distinct molecular signatures of cognitive change and C9ORF72 repeat expansion in the human ALS cortex

ZI Laszlo, N Hindley, A Sanchez Avila, RA Kline… - Acta neuropathologica …, 2022 - Springer
Increasing evidence suggests synaptic dysfunction is a central and possibly triggering factor
in Amyotrophic Lateral Sclerosis (ALS). Despite this, we still know very little about the …

Cell-autonomous requirement of TDP-43, an ALS/FTD signature protein, for oligodendrocyte survival and myelination

J Wang, WY Ho, K Lim, J Feng… - Proceedings of the …, 2018 - National Acad Sciences
TDP-43 aggregates in neurons and glia are the defining pathological hallmark of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), raising the …

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 …

Chronic BMAA exposure combined with TDP-43 mutation elicits motor neuron dysfunction phenotypes in mice

FJ Arnold, M Burns, Y Chiu, J Carvalho, AD Nguyen… - Neurobiology of …, 2023 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with an average age-of-
onset of∼ 60 years and is usually fatal within 2–5 years of diagnosis. Mouse models based …

Driven to decay: excitability and synaptic abnormalities in amyotrophic lateral sclerosis

MJ Fogarty - Brain research bulletin, 2018 - Elsevier
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron (MN) disease and is
clinically characterised by the death of corticospinal motor neurons (CSMNs), spinal and …

[HTML][HTML] Aging alters mechanisms underlying voluntary movements in spinal motor neurons of mice, primates, and humans

RW Castro, MC Lopes, RE Settlage, G Valdez - JCI insight, 2023 - ncbi.nlm.nih.gov
Spinal motor neurons have been implicated in the loss of motor function that occurs with
advancing age. However, the cellular and molecular mechanisms that impair the function of …