Mitochondrial dysfunction in ALS

M Cozzolino, MT Carrì - Progress in neurobiology, 2012 - Elsevier
In the present article, we review the many facets of mitochondrial dysfunction in amyotrophic
lateral sclerosis (ALS), a fatal neurodegenerative disease due to loss of upper motor …

SOD1 in ALS: taking stock in pathogenic mechanisms and the role of glial and muscle cells

C Peggion, V Scalcon, ML Massimino, K Nies… - Antioxidants, 2022 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
the loss of motor neurons in the brain and spinal cord. While the exact causes of ALS are still …

Skeletal muscle is a primary target of SOD1G93A-mediated toxicity

G Dobrowolny, M Aucello, E Rizzuto, S Beccafico… - Cell metabolism, 2008 - cell.com
The antioxidant enzyme superoxide dismutase 1 (SOD1) is a critical player of the
antioxidative defense whose activity is altered in several chronic diseases, including …

Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis

AU Joshi, NL Saw, H Vogel, AD Cunnigham… - EMBO molecular …, 2018 - embopress.org
Bioenergetic failure and oxidative stress are common pathological hallmarks of amyotrophic
lateral sclerosis (ALS), but whether these could be targeted effectively for novel therapeutic …

Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice

M Wong, LJ Martin - Human molecular genetics, 2010 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons
(MNs) that causes skeletal muscle paralysis. Familial forms of ALS are linked to mutations in …

Denervation-induced skeletal muscle atrophy is associated with increased mitochondrial ROS production

FL Muller, W Song, YC Jang, Y Liu… - American journal …, 2007 - journals.physiology.org
Reactive oxygen species (ROS), especially mitochondrial ROS, are postulated to play a
significant role in muscle atrophy. We report a dramatic increase in mitochondrial ROS …

The role of skeletal muscle in amyotrophic lateral sclerosis

JP Loeffler, G Picchiarelli, L Dupuis… - Brain …, 2016 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a fatal adult‐onset disease primarily characterized by
upper and lower motor neuron degeneration, muscle wasting and paralysis. It is increasingly …

Mitochondrial dysfunction and intracellular calcium dysregulation in ALS

H Kawamata, G Manfredi - Mechanisms of ageing and development, 2010 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder that affects
the aging population. A progressive loss of motor neurons in the spinal cord and brain leads …

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

R Mancuso, X Navarro - Progress in Neurobiology, 2015 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive degeneration of upper and lower motoneurons, leading to muscle weakness …

Mitochondrial respiratory chain dysfunction in muscle from patients with amyotrophic lateral sclerosis

V Crugnola, C Lamperti, V Lucchini… - Archives of …, 2010 - jamanetwork.com
Background Amyotrophic lateral sclerosis (ALS) is a major cause of neurological disability
and its pathogenesis remains elusive despite a multitude of studies. Although defects of the …