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

Reduced mitochondrial respiration and increased calcium deposits in the EDL muscle, but not in soleus, from 12-week-old dystrophic mdx mice

(2019) Rhayanna B. Gaglianone et al.   Scientific Reports

Duchenne muscular dystrophy awaits gene therapy

(2019) Dan Jones   NATURE BIOTECHNOLOGY

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

Mitochondria Bound to Lipid Droplets Have Unique Bioenergetics, Composition, and Dynamics that Support Lipid Droplet Expansion

(2018) Ilan Y. Benador et al.   Cell Metabolism

Estrogen receptor α protects pancreatic β-cells from apoptosis by preserving mitochondrial function and suppressing endoplasmic reticulum stress

(2018) Zhenqi Zhou et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Glucose Metabolism as a Pre-clinical Biomarker for the Golden Retriever Model of Duchenne Muscular Dystrophy

(2018) Sarah Morar Schneider et al.   MOLECULAR IMAGING AND BIOLOGY

Autophagy inhibition: a new therapeutic target in spinal muscular atrophy

(2018) Antonio Piras et al.   Neural Regeneration Research

Female dystrophinopathy: Review of current literature

(2018) Masatoshi Ishizaki et al.   NEUROMUSCULAR DISORDERS

Integrative effects of dystrophin loss on metabolic function of the mdx mouse

(2018) Jana Strakova et al.   Scientific Reports

Identification of Cardiomyopathy-Associated Circulating miRNA Biomarkers in Muscular Dystrophy Female Carriers Using a Complementary Cardiac Imaging and Plasma Profiling Approach

(2018) Anca Florian et al.   Frontiers in Physiology

The impact of exercise on mitochondrial dynamics and the role of Drp1 in exercise performance and training adaptations in skeletal muscle

(2018) Timothy M. Moore et al.   Molecular Metabolism

Skewed X-chromosome inactivation plays a crucial role in the onset of symptoms in carriers of Becker muscular dystrophy

(2017) Emanuela Viggiano et al.   JOURNAL OF GENE MEDICINE

Cardiac involvement in female carriers of Duchenne or Becker muscular dystrophy

(2017) Martin K. Childers et al.   MUSCLE & NERVE

Enhanced autophagy as a potential mechanism for the improved physiological function by simvastatin in muscular dystrophy

(2016) Nicholas P. Whitehead   Autophagy

Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy

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

A Single CRISPR-Cas9 Deletion Strategy that Targets the Majority of DMD Patients Restores Dystrophin Function in hiPSC-Derived Muscle Cells

(2016) Courtney S. Young et al.   Cell Stem Cell

PDGFRα signalling promotes fibrogenic responses in collagen-producing cells in Duchenne muscular dystrophy

(2016) Nicholas Ieronimakis et al.   JOURNAL OF PATHOLOGY

Skeletal muscle action of estrogen receptor α is critical for the maintenance of mitochondrial function and metabolic homeostasis in females

(2016) Vicent Ribas et al.   Science Translational Medicine

Mitochondrial dysfunctions during progression of dystrophic cardiomyopathy

(2015) Victoria Kyrychenko et al.   CELL CALCIUM

Autophagic flux determination in vivo and ex vivo

(2015) Lorena Esteban-Martínez et al.   METHODS

Cardiac involvement in female Duchenne and Becker muscular dystrophy carriers in comparison to their first-degree male relatives: a comparative cardiovascular magnetic resonance study

(2015) Anca Florian et al.   European Heart Journal-Cardiovascular Imaging

Muscular dystrophy in the mdx mouse is a severe myopathy compounded by hypotrophy, hypertrophy and hyperplasia

(2015) William Duddy et al.   Skeletal Muscle

Shaping the dynamic mitochondrial network

(2014) Laura L Lackner   BMC BIOLOGY

Early motor deficits in mouse disease models are reliably uncovered using an automated home-cage wheel-running system: a cross-laboratory validation

(2014) S. Mandillo et al.   Disease Models & Mechanisms

Skeletal Muscle Homeostasis in Duchenne Muscular Dystrophy: Modulating Autophagy as a Promising Therapeutic Strategy

(2014) Clara De Palma et al.   Frontiers in Aging Neuroscience

Post-natal induction of PGC-1α protects against severe muscle dystrophy independently of utrophin

(2014) Mun Chan et al.   Skeletal Muscle

The regulation of mitochondrial DNA copy number in glioblastoma cells

(2013) A Dickinson et al.   CELL DEATH AND DIFFERENTIATION

Ongoing therapeutic trials and outcome measures for Duchenne muscular dystrophy

(2013) Alessandra Govoni et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

HSP72 Is a Mitochondrial Stress Sensor Critical for Parkin Action, Oxidative Metabolism, and Insulin Sensitivity in Skeletal Muscle

(2013) Brian G. Drew et al.   DIABETES

Misregulation of autophagy and protein degradation systems in myopathies and muscular dystrophies

(2013) M. Sandri et al.   JOURNAL OF CELL SCIENCE

Mutations in Fis1 disrupt orderly disposal of defective mitochondria

(2013) Qinfang Shen et al.   MOLECULAR BIOLOGY OF THE CELL

Neuropsychological Profile of Duchenne Muscular Dystrophy

(2013) Anna Roshini Perumal et al.   Applied Neuropsychology-Child

Sarcospan: a small protein with large potential for Duchenne muscular dystrophy

(2013) Jamie L Marshall et al.   Skeletal Muscle

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

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

Genetic characterization in symptomatic female DMD carriers: lack of relationship between X-inactivation, transcriptional DMD allele balancing and phenotype

(2012) Simona Brioschi et al.   BMC Medical Genetics

E2F transcription factor-1 deficiency reduces pathophysiology in the mouse model of Duchenne muscular dystrophy through increased muscle oxidative metabolism

(2012) Emilie Blanchet et al.   HUMAN MOLECULAR GENETICS

Rescue of Dystrophic Skeletal Muscle by PGC-1α Involves a Fast to Slow Fiber Type Shift in the mdx Mouse

(2012) Joshua T. Selsby et al.   PLoS One

The Effects of Low Levels of Dystrophin on Mouse Muscle Function and Pathology

(2012) Maaike van Putten et al.   PLoS One

Autophagy as a new therapeutic target in Duchenne muscular dystrophy

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

Chronic Administration of a Leupeptin-Derived Calpain Inhibitor Fails to Ameliorate Severe Muscle Pathology in a Canine Model of Duchenne Muscular Dystrophy

(2012) Martin K. Childers et al.   Frontiers in Pharmacology

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

(2012) Vanessa E Jahnke et al.   Skeletal Muscle

Fusion and Fission: Interlinked Processes Critical for Mitochondrial Health

(2011) David C. Chan   Annual Review of Genetics

Characterization of macroautophagic flux in vivo using a leupeptin-based assay

(2011) Jeffrey Haspel et al.   Autophagy

Carrier Woman of Duchenne Muscular Dystrophy Mimicking Inflammatory Myositis

(2011) Jiyeol Yoon et al.   JOURNAL OF KOREAN MEDICAL SCIENCE

Current Status of Pharmaceutical and Genetic Therapeutic Approaches to Treat DMD

(2011) Christophe Pichavant et al.   MOLECULAR THERAPY

Leupeptin-based inhibitors do not improve the mdx phenotype

(2010) Joshua Selsby et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

The Interplay Between Mitochondrial Dynamics and Mitophagy

(2010) Gilad Twig et al.   ANTIOXIDANTS & REDOX SIGNALING

Metabolic control of mitochondrial biogenesis through the PGC-1 family regulatory network

(2010) Richard C. Scarpulla   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial Fusion Is Required for mtDNA Stability in Skeletal Muscle and Tolerance of mtDNA Mutations

(2010) Hsiuchen Chen et al.   CELL

Regulation of mitochondrial biogenesis

(2010) François R. Jornayvaz et al.   Essays in Biochemistry

New insights into the role of mitochondria in aging: mitochondrial dynamics and more

(2010) A. Y. Seo et al.   JOURNAL OF CELL SCIENCE

Mitochondrial DNA content and expression of genes involved in mtDNA transcription, regulation and maintenance during human fetal development

(2010) M. Pejznochova et al.   MITOCHONDRION

Circulating mitochondrial DAMPs cause inflammatory responses to injury

(2010) Qin Zhang et al.   NATURE

Mitochondrial fusion and fission in cell life and death

(2010) Benedikt Westermann   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

(2009) Katharine Bushby et al.   LANCET NEUROLOGY

Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care

(2009) Katharine Bushby et al.   LANCET NEUROLOGY

Mitochondrial Dynamics in Mammalian Health and Disease

(2009) Marc Liesa et al.   PHYSIOLOGICAL REVIEWS

Mitochondrial fusion, fission and autophagy as a quality control axis: The bioenergetic view

(2008) Gilad Twig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Cardiac involvement in a female carrier of Duchenne muscular dystrophy

(2008) Thomas Walcher et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

A Combined Metabolomic and Proteomic Investigation of the Effects of a Failure to Express Dystrophin in the Mouse Heart

(2008) Melanie K. Gulston et al.   JOURNAL OF PROTEOME RESEARCH

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

(2008) Douglas P Millay et al.   NATURE MEDICINE

Muscle Pain as the Only Presenting Symptom in a Girl With Dystrophinopathy

(2007) Berten P. Ceulemans et al.   PEDIATRIC NEUROLOGY