Treatment with a triazole inhibitor of the mitochondrial permeability transition pore fully corrects the pathology of sapje zebrafish lacking dystrophin

Molecular nature and regulation of the mitochondrial permeability transition pore(s), drug target(s) in cardioprotection

(2020) Michela Carraro et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Efficacy and safety of vamorolone in Duchenne muscular dystrophy: An 18-month interim analysis of a non-randomized open-label extension study

(2020) Edward C. Smith et al.   PLOS MEDICINE

Dystrophic mdx mouse myoblasts exhibit elevated ATP/UTP-evoked metabotropic purinergic responses and alterations in calcium signalling

(2019) Justyna Róg et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

Second‐Generation Inhibitors of the Mitochondrial Permeability Transition Pore with Improved Plasma Stability

(2019) Justina Šileikytė et al.   ChemMedChem

A novel class of cardioprotective small-molecule PTP inhibitors

(2019) Salvatore Antonucci et al.   PHARMACOLOGICAL RESEARCH

Novel procedures for whole organism detection and quantification of fluorescence as a measurement for oxidative stress in zebrafish ( Danio rerio ) larvae

(2018) Carina Lackmann et al.   CHEMOSPHERE

Gene and cell and therapy for muscular dystrophies: are we getting there?

(2018) Francesco Galli et al.   HUMAN GENE THERAPY

Dystrophin As a Molecular Shock Absorber

(2018) Shimin Le et al.   ACS Nano

Transient Opening of the Mitochondrial Permeability Transition Pore Induces Microdomain Calcium Transients in Astrocyte Processes

(2017) Amit Agarwal et al.   NEURON

Alisporivir rescues defective mitochondrial respiration in Duchenne muscular dystrophy

(2017) Marco Schiavone et al.   PHARMACOLOGICAL RESEARCH

Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy

(2017) Marshall W. Hogarth et al.   Nature Communications

Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy

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

P2Y2purinergic receptors are highly expressed in cardiac and diaphragm muscles ofmdxmice, and their expression is decreased by suramin

(2016) Drielen De Oliveira Moreira et al.   MUSCLE & NERVE

Forty years later: Mitochondria as therapeutic targets in muscle diseases

(2016) Alessandra Zulian et al.   PHARMACOLOGICAL RESEARCH

Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy

(2016) David G. Allen et al.   PHYSIOLOGICAL REVIEWS

The Pathogenesis and Therapy of Muscular Dystrophies

(2015) Simon Guiraud et al.   Annual Review of Genomics and Human Genetics

Store-operated calcium entry contributes to abnormal Ca2+ signalling in dystrophic mdx mouse myoblasts

(2015) Marta Onopiuk et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Spectrum of muscular dystrophies associated with sarcolemmal-protein genetic defects

(2015) Vincenzo Nigro et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Genetic evidence in the mouse solidifies the calcium hypothesis of myofiber death in muscular dystrophy

(2015) A R Burr et al.   CELL DEATH AND DIFFERENTIATION

Discovery, Synthesis, and Optimization of Diarylisoxazole-3-carboxamides as Potent Inhibitors of the Mitochondrial Permeability Transition Pore

(2015) Sudeshna Roy et al.   ChemMedChem

N-Phenylbenzamides as Potent Inhibitors of the Mitochondrial Permeability Transition Pore

(2015) Sudeshna Roy et al.   ChemMedChem

The Mitochondrial Permeability Transition Pore: Channel Formation by F-ATP Synthase, Integration in Signal Transduction, and Role in Pathophysiology

(2015) Paolo Bernardi et al.   PHYSIOLOGICAL REVIEWS

NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models

(2014) A. Zulian et al.   HUMAN MOLECULAR GENETICS

Efficient Mutagenesis by Cas9 Protein-Mediated Oligonucleotide Insertion and Large-Scale Assessment of Single-Guide RNAs

(2014) James A. Gagnon et al.   PLoS One

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

Analysis of Oxidative Stress in Zebrafish Embryos

(2014) Vera Mugoni et al.   Jove-Journal of Visualized Experiments

The Anticancer Drug Tamoxifen Counteracts the Pathology in a Mouse Model of Duchenne Muscular Dystrophy

(2013) Olivier M. Dorchies et al.   AMERICAN JOURNAL OF PATHOLOGY

Cyclophilin B is involved in p300-mediated degradation of CHOP in tumor cell adaptation to hypoxia

(2013) K Jeong et al.   CELL DEATH AND DIFFERENTIATION

ACTN3 genotype influences muscle performance through the regulation of calcineurin signaling

(2013) Jane T. Seto et al.   JOURNAL OF CLINICAL INVESTIGATION

Release of overexpressed CypB activates ERK signaling through CD147 binding for hepatoma cell resistance to oxidative stress

(2012) Kiyoon Kim et al.   APOPTOSIS

Quantification of birefringence readily measures the level of muscle damage in zebrafish

(2012) Joachim Berger et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Nitric Oxide in Myogenesis and Therapeutic Muscle Repair

(2012) Clara De Palma et al.   MOLECULAR NEUROBIOLOGY

Activation of the mitochondrial permeability transition pore modulates Ca2+ responses to physiological stimuli in adult neurons

(2011) Anna Barsukova et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

(2011) Luciano Merlini et al.   Oxidative Medicine and Cellular Longevity

δ-Sarcoglycan-deficient muscular dystrophy: from discovery to therapeutic approaches

(2011) Alison M Blain et al.   Skeletal Muscle

Immortalized pathological human myoblasts: towards a universal tool for the study of neuromuscular disorders

(2011) Kamel Mamchaoui et al.   Skeletal Muscle

Structural Basis for the Non-Immunosuppressive Character of the Cyclosporin A Analogue Debio 025

(2010) Isabelle Landrieu et al.   BIOCHEMISTRY

Zebrafish models of collagen VI-related myopathies

(2010) W.R. Telfer et al.   HUMAN MOLECULAR GENETICS

Cyclophilin D controls mitochondrial pore–dependent Ca2+ exchange, metabolic flexibility, and propensity for heart failure in mice

(2010) John W. Elrod et al.   JOURNAL OF CLINICAL INVESTIGATION

Debio-025 is more effective than prednisone in reducing muscular pathology in mdx mice

(2010) Erin R. Wissing et al.   NEUROMUSCULAR DISORDERS

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

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

Cyclophilin D in mitochondrial pathophysiology

(2009) Valentina Giorgio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects inCol6a1−/−myopathic mice

(2009) T Tiepolo et al.   BRITISH JOURNAL OF PHARMACOLOGY

Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice

(2009) Elena Palma et al.   HUMAN MOLECULAR GENETICS

Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle

(2009) Andrew M Bellinger et al.   NATURE MEDICINE

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

Calcium influx is sufficient to induce muscular dystrophy through a TRPC-dependent mechanism

(2009) Douglas P. Millay et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Postconditioning for salvage of ischemic skeletal muscle from reperfusion injury: efficacy and mechanism

(2008) Sandra E. McAllister et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy

(2008) J Reutenauer et al.   BRITISH JOURNAL OF PHARMACOLOGY

Cyclophilin A Mediates Vascular Remodeling by Promoting Inflammation and Vascular Smooth Muscle Cell Proliferation

(2008) Kimio Satoh et al.   CIRCULATION

Gene-mediated Restoration of Normal Myofiber Elasticity in Dystrophic Muscles

(2008) Stefania Puttini et al.   MOLECULAR THERAPY

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

(2008) Douglas P Millay et al.   NATURE MEDICINE

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

(2008) Luciano Merlini et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA