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 …

TDP-43 pathology: from noxious assembly to therapeutic removal

SS Keating, R San Gil, MEV Swanson, EL Scotter… - Progress in …, 2022 - Elsevier
Our understanding of amyotrophic lateral sclerosis and frontotemporal dementia has
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …

The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains

EG Conlon, L Lu, A Sharma, T Yamazaki, T Tang… - elife, 2016 - elifesciences.org
An expanded GGGGCC hexanucleotide in C9ORF72 (C9) is the most frequent known cause
of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). It has been …

From animal models to human disease: a genetic approach for personalized medicine in ALS

V Picher-Martel, PN Valdmanis, PV Gould… - Acta neuropathologica …, 2016 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is the most frequent motor neuron disease in
adults. Classical ALS is characterized by the death of upper and lower motor neurons …

Dendritic spine pathology in neurodegenerative diseases

J Herms, MM Dorostkar - Annual Review of Pathology …, 2016 - annualreviews.org
Substantial progress has been made toward understanding the neuropathology, genetic
origins, and epidemiology of neurodegenerative diseases, including Alzheimer's disease; …

Synaptic dysfunction in ALS and FTD: anatomical and molecular changes provide insights into mechanisms of disease

PA Gelon, PA Dutchak, CF Sephton - Frontiers in Molecular …, 2022 - frontiersin.org
Synaptic loss is a pathological feature of all neurodegenerative diseases including
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). ALS is a disease of …

[HTML][HTML] Impacts of exercise interventions on different diseases and organ functions in mice

S Guo, Y Huang, Y Zhang, H Huang, S Hong… - Journal of sport and health …, 2020 - Elsevier
Background In recent years, much evidence has emerged to indicate that exercise can
benefit people when performed properly. This review summarizes the exercise interventions …

Mouse models of ALS: Past, present and future

C Lutz - Brain Research, 2018 - Elsevier
Genome sequencing of both sporadic and familial patients of Amyotrophic Lateral Sclerosis
(ALS) has led to the identification of new genes that are both contributing and causative in …

Modelling amyotrophic lateral sclerosis in rodents

TW Todd, L Petrucelli - Nature Reviews Neuroscience, 2022 - nature.com
The efficient study of human disease requires the proper tools, one of the most crucial of
which is an accurate animal model that faithfully recapitulates the human condition. The …

Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia

L Renaud, V Picher-Martel, P Codron… - Acta neuropathologica …, 2019 - Springer
Abstract Ubiquilin-2 (UBQLN2) is a member of the ubiquilin family, actively implicated in the
degradation of misfolded and redundant proteins through the ubiquitin-proteasome system …