Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy

SR Casati, D Cervia, P Roux-Biejat, C Moscheni… - Cells, 2024 - mdpi.com
Duchenne muscular dystrophy (DMD) is a genetic progressive muscle-wasting disorder that
leads to rapid loss of mobility and premature death. The absence of functional dystrophin in …

[PDF][PDF] The role of mitochondria in Duchenne muscular dystrophy

M Budzinska, A Zimna, M Kurpisz - J Physiol Pharmacol, 2021 - jpp.krakow.pl
Duchenne muscular dystrophy (DMD) is an X-linked lethal disorder caused by mutations in
the dystrophin gene. Progression of this disease may lead to cardiomyopathy and …

The interplay of mitophagy and inflammation in Duchenne muscular dystrophy

AL Reid, MS Alexander - Life, 2021 - mdpi.com
Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease caused by a
pathogenic disruption of the DYSTROPHIN gene that results in non-functional dystrophin …

Early myopathy in Duchenne muscular dystrophy is associated with elevated mitochondrial H2O2 emission during impaired oxidative phosphorylation

MC Hughes, SV Ramos, PC Turnbull… - Journal of Cachexia …, 2019 - Wiley Online Library
Background Muscle wasting and weakness in Duchenne muscular dystrophy (DMD) causes
severe locomotor limitations and early death due in part to respiratory muscle failure. Given …

[HTML][HTML] Revisiting the dystrophin-ATP connection: How half a century of research still implicates mitochondrial dysfunction in Duchenne Muscular Dystrophy aetiology

CA Timpani, A Hayes, E Rybalka - Medical hypotheses, 2015 - Elsevier
Abstract Duchenne Muscular Dystrophy (DMD) is a fatal neuromuscular disease that is
characterised by dystrophin-deficiency and chronic Ca 2+-induced skeletal muscle wasting …

Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibition

JM Percival, MP Siegel, G Knowels… - Human molecular …, 2013 - academic.oup.com
Given the crucial roles for mitochondria in ATP energy supply, Ca2+ handling and cell
death, mitochondrial dysfunction has long been suspected to be an important pathogenic …

Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy

JH Kim, HB Kwak, LDV Thompson… - Journal of muscle research …, 2013 - Springer
Duchenne muscular dystrophy (DMD) is a degenerative skeletal muscle disease that makes
walking and breathing difficult. DMD is caused by an X-linked (Xp21) mutation in the …

Givinostat as metabolic enhancer reverting mitochondrial biogenesis deficit in Duchenne Muscular Dystrophy

M Giovarelli, S Zecchini, G Catarinella… - Pharmacological …, 2021 - Elsevier
Abstract Duchenne Muscular Dystrophy (DMD) is a rare disorder characterized by
progressive muscle wasting, weakness, and premature death. Remarkable progress has …

Mitochondrial stress responses in Duchenne muscular dystrophy: metabolic dysfunction or adaptive reprogramming?

CA Bellissimo, MC Garibotti… - American Journal of …, 2022 - journals.physiology.org
Mitochondrial stress may be a secondary contributor to muscle weakness in inherited
muscular dystrophies. Duchenne muscular dystrophy has received the majority of attention …

Mitochondrial creatine sensitivity is lost in the D2.mdx model of Duchenne muscular dystrophy and rescued by the mitochondrial-enhancing compound Olesoxime

CA Bellissimo, LJ Delfinis… - … of Physiology-Cell …, 2023 - journals.physiology.org
Duchenne muscular dystrophy (DMD) is associated with distinct mitochondrial stress
responses. Here, we aimed to determine whether the prospective mitochondrial-enhancing …