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

In vivo magnetic resonance spectroscopy in the brain of Cdkl5 null mice reveals a metabolic profile indicative of mitochondrial dysfunctions

(2021) Sara Carli et al.   JOURNAL OF NEUROCHEMISTRY

Biomarkers for Duchenne muscular dystrophy: myonecrosis, inflammation and oxidative stress

(2020) Miranda D. Grounds et al.   Disease Models & Mechanisms

HDAC inhibitors tune miRNAs in extracellular vesicles of dystrophic muscle‐resident mesenchymal cells

(2020) Martina Sandonà et al.   EMBO REPORTS

Global epidemiology of Duchenne muscular dystrophy: an updated systematic review and meta-analysis

(2020) Salvatore Crisafulli et al.   Orphanet Journal of Rare Diseases

Mitochondrial Dysfunction Is an Early Consequence of Partial or Complete Dystrophin Loss in mdx Mice

(2020) Timothy M. Moore et al.   Frontiers in Physiology

Early myopathy in Duchenne muscular dystrophy is associated with elevated mitochondrial H 2 O 2 emission during impaired oxidative phosphorylation

(2019) Meghan C. Hughes et al.   Journal of Cachexia Sarcopenia and Muscle

Diverse Action of Selected Statins on Skeletal Muscle Cells—An Attempt to Explain the Protective Effect of Geranylgeraniol (GGOH) in Statin-Associated Myopathy (SAM)

(2019) Anna Jaśkiewicz et al.   Journal of Clinical Medicine

Mitochondrial content is preserved throughout disease progression in the mdx mouse model of Duchenne muscular dystrophy, regardless of taurine supplementation

(2018) Robert G. Barker et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Effect of a long-term treatment with metformin in dystrophic mdx mice: A reconsideration of its potential clinical interest in Duchenne muscular dystrophy

(2018) Paola Mantuano et al.   BIOCHEMICAL PHARMACOLOGY

Autophagy balances mtDNA synthesis and degradation by DNA polymerase POLG during starvation

(2018) Tânia Catarina Medeiros et al.   JOURNAL OF CELL BIOLOGY

Proteome analysis in dystrophic mdx mouse muscle reveals a drastic alteration of key metabolic and contractile proteins after chronic exercise and the potential modulation by anti-oxidant compounds

(2018) Tania Gamberi et al.   Journal of Proteomics

Respiratory analysis of coupled mitochondria in cryopreserved liver biopsies

(2018) Mercedes García-Roche et al.   Redox Biology

Autophagy controls neonatal myogenesis by regulating the GH-IGF1 system through a NFE2L2- and DDIT3-mediated mechanism

(2018) Silvia Zecchini et al.   Autophagy

IP3 receptor blockade restores autophagy and mitochondrial function in skeletal muscle fibers of dystrophic mice

(2018) Denisse Valladares et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Alisporivir rescues defective mitochondrial respiration in Duchenne muscular dystrophy

(2017) Marco Schiavone et al.   PHARMACOLOGICAL RESEARCH

Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy

(2016) Maria C Vila et al.   CELL DEATH AND DIFFERENTIATION

Autophagy regulates satellite cell ability to regenerate normal and dystrophic muscles

(2016) E Fiacco et al.   CELL DEATH AND DIFFERENTIATION

Histological effects of givinostat in boys with Duchenne muscular dystrophy

(2016) Paolo Bettica et al.   NEUROMUSCULAR DISORDERS

Treatment effect of idebenone on inspiratory function in patients with Duchenne muscular dystrophy

(2016) Gunnar M. Buyse et al.   PEDIATRIC PULMONOLOGY

Reversal of Defective Mitochondrial Biogenesis in Limb-Girdle Muscular Dystrophy 2D by Independent Modulation of Histone and PGC-1α Acetylation

(2016) Sarah Pambianco et al.   Cell Reports

Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy

(2015) J. W. McGreevy et al.   Disease Models & Mechanisms

Efficacy of idebenone on respiratory function in patients with Duchenne muscular dystrophy not using glucocorticoids (DELOS): a double-blind randomised placebo-controlled phase 3 trial

(2015) Gunnar M Buyse et al.   LANCET

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

(2015) Cara A. Timpani et al.   MEDICAL HYPOTHESES

Muscle-specific Drp1 overexpression impairs skeletal muscle growth via translational attenuation

(2015) T Touvier et al.   Cell Death & Disease

Resveratrol induces expression of the slow, oxidative phenotype in mdx mouse muscle together with enhanced activity of the SIRT1-PGC-1α axis

(2014) Vladimir Ljubicic et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Gene expression in mdx mouse muscle in relation to age and exercise: aberrant mechanical–metabolic coupling and implications for pre-clinical studies in Duchenne muscular dystrophy

(2014) Giulia Maria Camerino et al.   HUMAN MOLECULAR GENETICS

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

(2014) Emma Rybalka et al.   PLoS One

Deficient nitric oxide signalling impairs skeletal muscle growth and performance: involvement of mitochondrial dysregulation

(2014) Clara De Palma et al.   Skeletal Muscle

The NAD+/Sirtuin Pathway Modulates Longevity through Activation of Mitochondrial UPR and FOXO Signaling

(2013) Laurent Mouchiroud et al.   CELL

Fibroadipogenic progenitors mediate the ability of HDAC inhibitors to promote regeneration in dystrophic muscles of young, but not old Mdx mice

(2013) Chiara Mozzetta et al.   EMBO Molecular Medicine

The therapeutic potential of skeletal muscle plasticity in Duchenne muscular dystrophy: phenotypic modifiers as pharmacologic targets

(2013) Vladimir Ljubicic et al.   FASEB JOURNAL

Skeletal muscle mitochondrial and lipid droplet content assessed with standardized grid sizes for stereology

(2013) Nicholas T. Broskey et al.   JOURNAL OF APPLIED PHYSIOLOGY

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(2013) Silvia Consalvi et al.   MOLECULAR MEDICINE

Epigenetic Reprogramming of Human Embryonic Stem Cells into Skeletal Muscle Cells and Generation of Contractile Myospheres

(2013) Sonia Albini et al.   Cell Reports

AMPK Activation Stimulates Autophagy and Ameliorates Muscular Dystrophy in the mdx Mouse Diaphragm

(2012) Marion Pauly et al.   AMERICAN JOURNAL OF PATHOLOGY

An efficient extraction method for quantitation of adenosine triphosphate in mammalian tissues and cells

(2012) Junji Chida et al.   ANALYTICA CHIMICA ACTA

Mitochondrial function in human skeletal muscle following high-altitude exposure

(2012) Robert A. Jacobs et al.   EXPERIMENTAL PHYSIOLOGY

Mouse lines with photo-activatable mitochondria to study mitochondrial dynamics

(2012) Anh H. Pham et al.   GENESIS

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

(2012) J. M. Percival et al.   HUMAN MOLECULAR GENETICS

Peroxisome proliferator-activated receptor γ coactivator 1-α gene transfer restores mitochondrial biomass and improves mitochondrial calcium handling in post-necroticmdxmouse skeletal muscle

(2012) Richard Godin et al.   JOURNAL OF PHYSIOLOGY-LONDON

Autophagy as a new therapeutic target in Duchenne muscular dystrophy

(2012) C De Palma et al.   Cell Death & Disease

Metabolic remodeling agents show beneficial effects in the dystrophin-deficient mdx mouse model

(2012) Vanessa E Jahnke et al.   Skeletal Muscle

Chronic AMPK stimulation attenuates adaptive signaling in dystrophic skeletal muscle

(2011) Vladimir Ljubicic et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Safety and efficacy of an oral histone deacetylase inhibitor in systemic-onset juvenile idiopathic arthritis

(2011) Jelena Vojinovic et al.   ARTHRITIS AND RHEUMATISM

Defects in mitochondrial DNA replication and human disease

(2011) William C. Copeland   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Chronic AMPK activation evokes the slow, oxidative myogenic program and triggers beneficial adaptations in mdx mouse skeletal muscle

(2011) V. Ljubicic et al.   HUMAN MOLECULAR GENETICS

Stage of perinatal development regulates skeletal muscle mitochondrial biogenesis and myogenic regulatory factor genes with little impact of growth restriction or cross-fostering

(2011) R. C. Laker et al.   Journal of Developmental Origins of Health and Disease

Nitric oxide inhibition of Drp1-mediated mitochondrial fission is critical for myogenic differentiation

(2010) C De Palma et al.   CELL DEATH AND DIFFERENTIATION

Mitochondrial respiratory chain and creatine kinase activities inmdxmouse brain

(2010) Lisiane Tuon et al.   MUSCLE & NERVE

Mitochondrial DNA depletion syndromes – Many genes, common mechanisms

(2010) Anu Suomalainen et al.   NEUROMUSCULAR DISORDERS

Transcriptional Control of Mitochondrial Biogenesis and Function

(2009) M. Benjamin Hock et al.   Annual Review of Physiology

Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts

(2009) Marta Onopiuk et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Pharmacological activation of PPAR /  stimulates utrophin A expression in skeletal muscle fibers and restores sarcolemmal integrity in mature mdx mice

(2009) P. Miura et al.   HUMAN MOLECULAR GENETICS

Multiple pathological events in exercised dystrophic mdx mice are targeted by pentoxifylline: outcome of a large array of in vivo and ex vivo tests

(2009) Rosa Burdi et al.   JOURNAL OF APPLIED PHYSIOLOGY

Reciprocal amplification of ROS and Ca2+ signals in stressed mdx dystrophic skeletal muscle fibers

(2009) Vyacheslav M. Shkryl et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Mechanisms of Muscle Degeneration, Regeneration, and Repair in the Muscular Dystrophies

(2008) Gregory Q. Wallace et al.   Annual Review of Physiology

Abnormal cardiac morphology, function and energy metabolism in the dystrophic mdx mouse: An MRI and MRS study

(2008) Wen Zhang et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy

(2008) Douglas P Millay et al.   NATURE MEDICINE

HDAC2 blockade by nitric oxide and histone deacetylase inhibitors reveals a common target in Duchenne muscular dystrophy treatment

(2008) C. Colussi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA