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 …

Bi-phasic dose response in the preclinical and clinical developments of sigma-1 receptor ligands for the treatment of neurodegenerative disorders

T Maurice - Expert Opinion on Drug Discovery, 2021 - Taylor & Francis
ABSTRACT Introduction: The sigma-1 receptor (S1R) is attracting much attention for disease-
modifying therapies in neurodegenerative diseases. It is a conserved protein, present in …

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

SA Davis, S Itaman, CM Khalid-Janney, JA Sherard… - Neuroscience …, 2018 - Elsevier
Transactive response DNA-binding protein of 43 kDa (TDP-43) functions as a
heterogeneous nuclear ribonucleoprotein and is the major pathological protein in …

Glycolysis upregulation is neuroprotective as a compensatory mechanism in ALS

E Manzo, I Lorenzini, D Barrameda, AG O'Conner… - Elife, 2019 - elifesciences.org
Amyotrophic Lateral Sclerosis (ALS), is a fatal neurodegenerative disorder, with TDP-43
inclusions as a major pathological hallmark. Using a Drosophila model of TDP-43 …

The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B-mediated inflammatory response

S Lee, S Kim, HY Kang, HR Lim, Y Kwon, M Jo… - Journal of …, 2020 - Springer
Background Cytoplasmic inclusions of transactive response DNA binding protein of 43 kDa
(TDP-43) in neurons and astrocytes are a feature of some neurodegenerative diseases …

Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges

L Zhu, S Li, XJ Li, P Yin - Translational neurodegeneration, 2023 - Springer
In order to dissect amyotrophic lateral sclerosis (ALS), a multigenic, multifactorial, and
progressive neurodegenerative disease with heterogeneous clinical presentations …

mRNP assembly, axonal transport, and local translation in neurodegenerative diseases

B Khalil, D Morderer, PL Price, F Liu, W Rossoll - Brain Research, 2018 - Elsevier
The development, maturation, and maintenance of the mammalian nervous system rely on
complex spatiotemporal patterns of gene expression. In neurons, this is achieved by the …

Fly for ALS: Drosophila modeling on the route to amyotrophic lateral sclerosis modifiers

F Liguori, S Amadio, C Volonté - Cellular and Molecular Life Sciences, 2021 - Springer
Amyotrophic lateral sclerosis (ALS) is a rare, devastating disease, causing movement
impairment, respiratory failure and ultimate death. A plethora of genetic, cellular and …

[HTML][HTML] Altered bioenergetics and metabolic homeostasis in amyotrophic lateral sclerosis

AT Nelson, D Trotti - Neurotherapeutics, 2022 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that
primarily affects motor neurons and causes muscle atrophy, paralysis, and death. While a …

Mitochondrial quality control strategies: potential therapeutic targets for neurodegenerative diseases?

D Hu, Z Liu, X Qi - Frontiers in Neuroscience, 2021 - frontiersin.org
Many lines of evidence have indicated the therapeutic potential of rescuing mitochondrial
integrity by targeting specific mitochondrial quality control pathways in neurodegenerative …