Mitochondrial Cardiomyopathy: Molecular Epidemiology, Diagnosis, Models, and Therapeutic Management

Recent Advances in Modeling Mitochondrial Cardiomyopathy Using Human Induced Pluripotent Stem Cells

(2022) Mario G. Pavez-Giani et al.   Frontiers in Cell and Developmental Biology

Mitochondrial transfer/transplantation: an emerging therapeutic approach for multiple diseases

(2022) Zonghan Liu et al.   Cell and Bioscience

Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy

(2022) Nicole M. Sayles et al.   Cell Reports

Mitochondrial Transfer Improves Cardiomyocyte Bioenergetics and Viability in Male Rats Exposed to Pregestational Diabetes

(2021) Eli J. Louwagie et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondrial transplantation in cardiomyocytes: foundation, methods, and outcomes

(2021) Paria Ali Pour et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy

(2021) Siting Zhu et al.   Circulation-Heart Failure

Stomatin-Like Protein-2: A Potential Target to Treat Mitochondrial Cardiomyopathy

(2021) Yuntao Hu et al.   Heart Lung and Circulation

Long-term prognosis and genetic background of cardiomyopathy in 223 pediatric mitochondrial disease patients

(2021) Atsuko Imai-Okazaki et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Human induced pluripotent stem cells for modelling metabolic perturbations and impaired bioenergetics underlying cardiomyopathies

(2020) Chrishan J A Ramachandra et al.   CARDIOVASCULAR RESEARCH

Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations

(2020) Yi-Ting Liu et al.   HUMAN MOLECULAR GENETICS

Bioenergetics Consequences of Mitochondrial Transplantation in Cardiomyocytes

(2020) Paria Ali Pour et al.   Journal of the American Heart Association

Generation of two induced pluripotent stem cell lines (XACHi0010-A, XACHi0011-A) from a Chinese family with combined oxidative phosphorylation deficiency carrying homozygous and heterozygous C1QBP-L275F mutation

(2020) Yanmin Zhang et al.   Stem Cell Research

ndufa7 plays a critical role in cardiac hypertrophy

(2020) Xingjuan Shi et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Cardiomyopathy in children with mitochondrial disease: Prognosis and genetic background

(2019) Atsuko Imai-Okazaki et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Mitochondrial DNA Integrity: Role in Health and Disease

(2019) Priyanka Sharma et al.   Cells

A QIL1 Variant Associated with Ventricular Arrhythmias and Sudden Cardiac Death in the Juvenile Rhodesian Ridgeback Dog

(2019) Kathryn Meurs et al.   Genes

Mesenchymal stem cells and their mitochondrial transfer: a double-edged sword

(2019) Cheng Li et al.   BIOSCIENCE REPORTS

Modelling diastolic dysfunction in induced pluripotent stem cell-derived cardiomyocytes from hypertrophic cardiomyopathy patients

(2019) Haodi Wu et al.   EUROPEAN HEART JOURNAL

Mitochondrial transplantation ameliorates acute limb ischemia

(2019) Arzoo Orfany et al.   JOURNAL OF VASCULAR SURGERY

Vascular Inflammation and Oxidative Stress: Major Triggers for Cardiovascular Disease

(2019) Sebastian Steven et al.   Oxidative Medicine and Cellular Longevity

Delayed Transplantation of Autologous Mitochondria for Cardioprotection in a Porcine Model

(2019) David Blitzer et al.   ANNALS OF THORACIC SURGERY

Reversible Mitochondrial Fragmentation in iPSC-Derived Cardiomyocytes from Children with DCMA, a Mitochondrial Cardiomyopathy

(2019) Leili Rohani et al.   CANADIAN JOURNAL OF CARDIOLOGY

Mitochondrial transplantation for myocardial protection in diabetic hearts

(2019) Ilias P Doulamis et al.   EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY

Therapeutic use of extracellular mitochondria in CNS injury and disease

(2019) Yoshihiko Nakamura et al.   EXPERIMENTAL NEUROLOGY

Loss of tafazzin results in decreased myoblast differentiation in C2C12 cells: A myoblast model of Barth syndrome and cardiolipin deficiency

(2018) Wenjia Lou et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Mitochondrial function in the heart: The insight into mechanisms and therapeutic potentials

(2018) Binh Yen Nguyen et al.   BRITISH JOURNAL OF PHARMACOLOGY

Advances in methods for reducing mitochondrial DNA disease by replacing or manipulating the mitochondrial genome

(2018) Pavandeep K. Rai et al.   Essays in Biochemistry

MitoQ improves mitochondrial dysfunction in heart failure induced by pressure overload

(2018) Rogério Faustino Ribeiro Junior et al.   FREE RADICAL BIOLOGY AND MEDICINE

Chronic Supplementation With a Mitochondrial Antioxidant (MitoQ) Improves Vascular Function in Healthy Older AdultsNovelty and Significance

(2018) Matthew J. Rossman et al.   HYPERTENSION

Mitochondrial Dysfunctions Contribute to Hypertrophic Cardiomyopathy in Patient iPSC-Derived Cardiomyocytes with MT-RNR2 Mutation

(2018) Shishi Li et al.   Stem Cell Reports

What the cardiologist should know about mitochondrial cardiomyopathy ?

(2018) Guillaume St-Pierre et al.   CANADIAN JOURNAL OF CARDIOLOGY

Alternative oxidase‐mediated respiration prevents lethal mitochondrial cardiomyopathy

(2018) Jayasimman Rajendran et al.   EMBO Molecular Medicine

Connexin 43-Mediated Mitochondrial Transfer of iPSC-MSCs Alleviates Asthma Inflammation

(2018) Yin Yao et al.   Stem Cell Reports

Autologous mitochondrial transplantation for dysfunction after ischemia-reperfusion injury

(2017) Sitaram M. Emani et al.   JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY

Mitochondrial Transfer from Wharton’s Jelly Mesenchymal Stem Cell to MERRF Cybrid Reduces Oxidative Stress and Improves Mitochondrial Bioenergetics

(2017) Yao-Chung Chuang et al.   Oxidative Medicine and Cellular Longevity

Regenerative Medicine/Cardiac Cell Therapy: Pluripotent Stem Cells

(2017) Ana Duran et al.   THORACIC AND CARDIOVASCULAR SURGEON

Mitochondrial respiratory dysfunction disturbs neuronal and cardiac lineage commitment of human iPSCs

(2017) Mutsumi Yokota et al.   Cell Death & Disease

Metabolic stress-induced cardiomyopathy is caused by mitochondrial dysfunction due to attenuated Erk5 signaling

(2017) Wei Liu et al.   Nature Communications

Mitotherapy for Fatty Liver by Intravenous Administration of Exogenous Mitochondria in Male Mice

(2017) Ailing Fu et al.   Frontiers in Pharmacology

Nicotinamide Riboside Preserves Cardiac Function in a Mouse Model of Dilated Cardiomyopathy

(2017) Nicolas Diguet et al.   CIRCULATION

Mitochondrial Cardiomyopathy Caused by Elevated Reactive Oxygen Species and Impaired Cardiomyocyte Proliferation

(2017) Donghui Zhang et al.   CIRCULATION RESEARCH

Superoxide dismutase/catalase mimetic EUK-134 prevents diaphragm muscle weakness in monocrotalin-induced pulmonary hypertension

(2017) Koichi Himori et al.   PLoS One

Mitochondrial Transfer by Photothermal Nanoblade Restores Metabolite Profile in Mammalian Cells

(2016) Ting-Hsiang Wu et al.   Cell Metabolism

Transferring Xenogenic Mitochondria Provides Neural Protection against Ischemic Stress in Ischemic Rat Brains

(2016) Po-Jui Huang et al.   CELL TRANSPLANTATION

Mitochondrial involvement in myocyte death and heart failure

(2016) Michael J. Goldenthal   HEART FAILURE REVIEWS

Mouse models for mitochondrial diseases

(2016) Benedetta Ruzzenente et al.   HUMAN MOLECULAR GENETICS

Towards clinical application of pronuclear transfer to prevent mitochondrial DNA disease

(2016) Louise A. Hyslop et al.   NATURE

Mitochondrial function as a therapeutic target in heart failure

(2016) David A. Brown et al.   Nature Reviews Cardiology

Allogeneic/xenogeneic transplantation of peptide-labeled mitochondria in Parkinson's disease: restoration of mitochondria functions and attenuation of 6-hydroxydopamine–induced neurotoxicity

(2016) Jui-Chih Chang et al.   Translational Research

Mitochondrial transplantation attenuates hypoxic pulmonary vasoconstriction

(2016) Juan Zhou et al.   Oncotarget

A Comprehensive Genomic Analysis Reveals the Genetic Landscape of Mitochondrial Respiratory Chain Complex Deficiencies

(2016) Masakazu Kohda et al.   PLoS Genetics

iPSC-MSCs with High Intrinsic MIRO1 and Sensitivity to TNF-α Yield Efficacious Mitochondrial Transfer to Rescue Anthracycline-Induced Cardiomyopathy

(2016) Yuelin Zhang et al.   Stem Cell Reports

Mitochondrial Diseases and Cardiomyopathies

(2015) Catherine Brunel-Guitton et al.   CANADIAN JOURNAL OF CARDIOLOGY

Mitochondria-targeted antioxidant preserves contractile properties and mitochondrial function of skeletal muscle in aged rats

(2015) Sabzali Javadov et al.   Oncotarget

Mutation in MRPS34 Compromises Protein Synthesis and Causes Mitochondrial Dysfunction

(2015) Tara R. Richman et al.   PLoS Genetics

Impaired respiratory function in MELAS-induced pluripotent stem cells with high heteroplasmy levels

(2015) Masaki Kodaira et al.   FEBS Open Bio

Mitochondrial Transfer of Induced Pluripotent Stem Cell–Derived Mesenchymal Stem Cells to Airway Epithelial Cells Attenuates Cigarette Smoke–Induced Damage

(2014) Xiang Li et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Extracellular Volume Imaging and Quantitative T2 Mapping for the Diagnosis of Mitochondrial Cardiomyopathy

(2014) Kyoung Hwa Lee et al.   CIRCULATION

Cardiac manifestations of primary mitochondrial disorders

(2014) Josef Finsterer et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Serum supplemented culture medium masks hypertrophic phenotypes in human pluripotent stem cell derived cardiomyocytes

(2014) Cheryl Dambrot et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

(2014) Morgane Perdomini et al.   NATURE MEDICINE

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies

(2014) Gang Wang et al.   NATURE MEDICINE

Modeling of Friedreich ataxia-related iron overloading cardiomyopathy using patient-specific-induced pluripotent stem cells

(2013) Yee-Ki Lee et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Cardiomyocyte Specific Deletion of Crif1 Causes Mitochondrial Cardiomyopathy in Mice

(2013) Juhee Shin et al.   PLoS One

Erosion of Dosage Compensation Impacts Human iPSC Disease Modeling

(2012) Shila Mekhoubad et al.   Cell Stem Cell

Cardiac involvement in mitochondrial DNA disease: clinical spectrum, diagnosis, and management

(2012) M. G. D. Bates et al.   EUROPEAN HEART JOURNAL

Induced Pluripotent Mesenchymal Stromal Cell Clones Retain Donor-derived Differences in DNA Methylation Profiles

(2012) Kaifeng Shao et al.   MOLECULAR THERAPY

Towards germline gene therapy of inherited mitochondrial diseases

(2012) Masahito Tachibana et al.   NATURE

Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy

(2012) B.-X. Ke et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Correction of the consequences of mitochondrial 3243A>G mutation in the MT-TL1 gene causing the MELAS syndrome by tRNA import into mitochondria

(2011) O. Z. Karicheva et al.   NUCLEIC ACIDS RESEARCH

Lethal mitochondrial cardiomyopathy in a hypomorphic Med30 mouse mutant is ameliorated by ketogenic diet

(2011) P. Krebs et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pluripotent stem cell models of cardiac disease and their implication for drug discovery and development

(2011) Richard P. Davis et al.   TRENDS IN MOLECULAR MEDICINE

Adenine Nucleotide Translocase 1 Deficiency Results in Dilated Cardiomyopathy With Defects in Myocardial Mechanics, Histopathological Alterations, and Activation of Apoptosis

(2011) Nupoor Narula et al.   JACC-Cardiovascular Imaging

Therapeutic Targeting of Mitochondrial Superoxide in Hypertension

(2010) Anna E. Dikalova et al.   CIRCULATION RESEARCH

Patient-Specific Induced Pluripotent Stem-Cell Models for Long-QT Syndrome

(2010) Alessandra Moretti et al.   NEW ENGLAND JOURNAL OF MEDICINE

CMR gives clue to “ragged red fibers” in the heart in a patient with mitochondrial myopathy

(2009) Tania Jose et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY