ERK1/2-dependent TSPO overactivation associates with the loss of mitophagy and mitochondrial respiration in ALS

A Magrì, CLR Lipari, P Risiglione, S Zimbone… - Cell Death & …, 2023 - nature.com
Mitochondrial dysfunction and the loss of mitophagy, aimed at recycling irreversibly
damaged organelles, contribute to the onset of amyotrophic lateral sclerosis (ALS), a fatal …

Mitochondrial quality control in amyotrophic lateral sclerosis: towards a common pathway?

B Khalil, JC Liévens - Neural Regeneration Research, 2017 - journals.lww.com
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder
characterized by loss of upper and lower motor neurons. Different mechanisms contribute to …

Mitochondria in amyotrophic lateral sclerosis: a trigger and a target

L Dupuis, JL Gonzalez de Aguilar, H Oudart… - Neurodegenerative …, 2005 - karger.com
Strong evidence shows that mitochondrial dysfunction is involved in amyotrophic lateral
sclerosis (ALS), but despite the fact that mitochondria play a central role in excitotoxicity …

ASCs-Exosomes Recover Coupling Efficiency and Mitochondrial Membrane Potential in an in vitro Model of ALS

E Calabria, I Scambi, R Bonafede… - Frontiers in …, 2019 - frontiersin.org
The amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized
by motoneurons death. Mutations in the superoxide dismutase 1 (SOD1) protein have been …

[HTML][HTML] Mitochondrial dysfunction in amyotrophic lateral sclerosis

P Shi, J Gal, DM Kwinter, X Liu, H Zhu - Biochimica et Biophysica Acta (BBA …, 2010 - Elsevier
The etiology of motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remains to
be better understood. Based on the studies from ALS patients and transgenic animal …

Mitochondrial dysfunction is a converging point of multiple pathological pathways in amyotrophic lateral sclerosis

P Shi, Y Wei, J Zhang, J Gal… - Journal of Alzheimer's …, 2010 - content.iospress.com
A better understanding of the etiology of amyotrophic lateral sclerosis (ALS) is needed to
develop effective therapies for the treatment of this fatal neurodegenerative disease …

ROS-related mitochondrial dysfunction in skeletal muscle of an ALS mouse model during the disease progression

Y Xiao, C Karam, J Yi, L Zhang, X Li, D Yoon… - Pharmacological …, 2018 - Elsevier
In amyotrophic lateral sclerosis (ALS), mitochondrial dysfunction and oxidative stress form a
vicious cycle that promotes neurodegeneration and muscle wasting. To quantify the disease …

Mitochondrial dysfunction and its role in motor neuron degeneration in ALS

G Manfredi, Z Xu - Mitochondrion, 2005 - Elsevier
Mitochondria play a pivotal role in many metabolic and apoptotic pathways that regulate the
life and death of cells. Accumulating evidence suggests that mitochondrial dysfunction is …

Parkin is a disease modifier in the mutant SOD 1 mouse model of ALS

GM Palomo, V Granatiero, H Kawamata… - EMBO molecular …, 2018 - embopress.org
Abstract Mutant Cu/Zn superoxide dismutase (SOD 1) causes mitochondrial alterations that
contribute to motor neuron demise in amyotrophic lateral sclerosis (ALS). When …

[HTML][HTML] Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis

W Tan, P Pasinelli, D Trotti - … et Biophysica Acta (BBA)-Molecular Basis of …, 2014 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with an adult onset
characterized by loss of both upper and lower motor neurons. In~ 10% of cases, patients …