Duchenne muscular dystrophy: Disease mechanism and therapeutic strategies

A Bez Batti Angulski, N Hosny, H Cohen… - Frontiers in …, 2023 - frontiersin.org
Duchenne muscular dystrophy (DMD) is a severe, progressive, and ultimately fatal disease
of skeletal muscle wasting, respiratory insufficiency, and cardiomyopathy. The identification …

Osteoporosis, genetics and hormones

R Rizzoli, JP Bonjour, SL Ferrari - Journal of molecular …, 2001 - jme.bioscientifica.com
Currently, there is no completely effective therapy available for metastatic
phaeochromocytomas (PCCs) and paragangliomas. In this study, we explore new molecular …

Analysis and interpretation of microplate-based oxygen consumption and pH data

AS Divakaruni, A Paradyse, DA Ferrick, AN Murphy… - Methods in …, 2014 - Elsevier
Breakthrough technologies to measure cellular oxygen consumption and proton efflux are
reigniting the study of cellular energetics by increasing the scope and pace with which …

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 …

Tissue-engineered skeletal muscle models to study muscle function, plasticity, and disease

A Khodabukus - Frontiers in Physiology, 2021 - frontiersin.org
Skeletal muscle possesses remarkable plasticity that permits functional adaptations to a
wide range of signals such as motor input, exercise, and disease. Small animal models have …

Methods for assessing mitochondrial function in diabetes

CGR Perry, DA Kane, IR Lanza, PD Neufer - Diabetes, 2013 - Am Diabetes Assoc
A growing body of research is investigating the potential contribution of mitochondrial
function to the etiology of type 2 diabetes. Numerous in vitro, in situ, and in vivo …

Mitochondrial dysfunction in skeletal muscle contributes to the development of acute insulin resistance in mice

H Lee, TY Ha, CH Jung, FS Nirmala… - Journal of Cachexia …, 2021 - Wiley Online Library
Background Although mounting evidence indicates that insulin resistance (IR) co‐occurs
with mitochondrial dysfunction in skeletal muscle, there is no clear causal link between …

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 …

[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 ATP Synthesis in Dystrophin-Deficient Mdx Skeletal Muscles May Be Caused by Complex I Insufficiency

E Rybalka, CA Timpani, MB Cooke, AD Williams… - PloS one, 2014 - journals.plos.org
Duchenne Muscular Dystrophy is a chronic, progressive and ultimately fatal skeletal muscle
wasting disease characterised by sarcolemmal fragility and intracellular Ca2+ dysregulation …