Neuronal hyperexcitability in Alzheimer's disease: what are the drivers behind this aberrant phenotype?

H Targa Dias Anastacio, N Matosin, L Ooi - Translational psychiatry, 2022 - nature.com
Alzheimer's disease (AD) is a progressive neurodegenerative disorder leading to loss of
cognitive abilities and ultimately, death. With no cure available, limited treatments mostly …

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 …

Antisense oligonucleotide silencing of FUS expression as a therapeutic approach in amyotrophic lateral sclerosis

VA Korobeynikov, AK Lyashchenko… - Nature medicine, 2022 - nature.com
Fused in sarcoma (FUS) is an RNA-binding protein that is genetically and pathologically
associated with rare and aggressive forms of amyotrophic lateral sclerosis (ALS) and …

Single-cell profiling of the developing mouse brain and spinal cord with split-pool barcoding

AB Rosenberg, CM Roco, RA Muscat, A Kuchina… - Science, 2018 - science.org
To facilitate scalable profiling of single cells, we developed split-pool ligation-based
transcriptome sequencing (SPLiT-seq), a single-cell RNA-seq (scRNA-seq) method that …

Single nucleus RNA-sequencing defines unexpected diversity of cholinergic neuron types in the adult mouse spinal cord

MR Alkaslasi, ZE Piccus, S Hareendran… - Nature …, 2021 - nature.com
In vertebrates, motor control relies on cholinergic neurons in the spinal cord that have been
extensively studied over the past hundred years, yet the full heterogeneity of these neurons …

Muscle spindle function in healthy and diseased muscle

S Kröger, B Watkins - Skeletal Muscle, 2021 - Springer
Almost every muscle contains muscle spindles. These delicate sensory receptors inform the
central nervous system (CNS) about changes in the length of individual muscles and the …

Dynamic neuromuscular remodeling precedes motor-unit loss in a mouse model of ALS

É Martineau, A Di Polo, C Vande Velde, R Robitaille - Elife, 2018 - elifesciences.org
Despite being an early event in ALS, it remains unclear whether the denervation of
neuromuscular junctions (NMJ) is simply the first manifestation of a globally degenerating …

Pathologically mislocalised TDP-43 in upper motor neurons causes a die-forward spread of ALS-like pathogenic changes throughout the mouse corticomotor system

LA Reale, MS Dyer, SE Perry, KM Young… - Progress in …, 2023 - Elsevier
Alterations in upper motor neuron excitability are one of the earliest phenomena clinically
detected in ALS, and in 97% of cases, the RNA/DNA binding protein, TDP-43, is …

Proteostatic imbalance and protein spreading in amyotrophic lateral sclerosis

ME Cicardi, L Marrone, M Azzouz, D Trotti - The EMBO journal, 2021 - embopress.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder whose exact
causative mechanisms are still under intense investigation. Several lines of evidence …

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 …