Metabolic rescue ameliorates mitochondrial encephalo‐cardiomyopathy in murine and human iPSC models of Leigh syndrome

Hypoxia ameliorates brain hyperoxia and NAD+ deficiency in a murine model of Leigh syndrome

(2021) Robert M.H. Grange et al.   MOLECULAR GENETICS AND METABOLISM

Modulation of the cardiac sodium channel NaV1.5 peak and late currents by NAD+ precursors

(2020) Daniel S. Matasic et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Extreme Acetylation of the Cardiac Mitochondrial Proteome Does Not Promote Heart Failure

(2020) Michael Thomas Davidson et al.   CIRCULATION RESEARCH

Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease

(2020) Simon C. Johnson et al.   MOLECULAR GENETICS AND METABOLISM

Adult mesenchymal stem cell ageing interplays with depressed mitochondrial Ndufs6

(2020) Yuelin Zhang et al.   Cell Death & Disease

Maresin 1 attenuates the inflammatory response and mitochondrial damage in mice with cerebral ischemia/reperfusion in a SIRT1-dependent manner

(2019) Wenjing Xian et al.   BRAIN RESEARCH

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

(2019) Chi Fung Lee et al.   Scientific Reports

Role of SIRT1 in Modulating Acetylation of the Sarco-Endoplasmic Reticulum Ca 2+ -ATPase in Heart Failure

(2019) Przemyslaw A Gorski et al.   CIRCULATION RESEARCH

Safety and Metabolism of Long-term Administration of NIAGEN (Nicotinamide Riboside Chloride) in a Randomized, Double-Blind, Placebo-controlled Clinical Trial of Healthy Overweight Adults

(2019) Dietrich Conze et al.   Scientific Reports

Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation

(2019) Isha H. Jain et al.   Cell Metabolism

Management of Leigh syndrome: Current status and new insights

(2018) L. Chen et al.   CLINICAL GENETICS

Heart specific knockout of Ndufs4 ameliorates ischemia reperfusion injury

(2018) Huiliang Zhang et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

mTOR inhibitors may benefit kidney transplant recipients with mitochondrial diseases

(2018) Simon C. Johnson et al.   KIDNEY INTERNATIONAL

Role of protein kinase C in metabolic regulation of the cardiac Na+ channel

(2017) Man Liu et al.   HEART RHYTHM

Tachycardia-bradycardia syndrome: Electrophysiological mechanisms and future therapeutic approaches (Review)

(2017) Gary Tse et al.   INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE

Sirtuin 1 regulates cardiac electrical activity by deacetylating the cardiac sodium channel

(2017) Ajit Vikram et al.   NATURE MEDICINE

NOREVA: normalization and evaluation of MS-based metabolomics data

(2017) Bo Li et al.   NUCLEIC ACIDS RESEARCH

Hypoxia as a therapy for mitochondrial disease

(2016) I. H. Jain et al.   SCIENCE

Nicotinamide riboside is uniquely and orally bioavailable in mice and humans

(2016) Samuel A. J. Trammell et al.   Nature Communications

Mitochondrial Complex I Activity Suppresses Inflammation and Enhances Bone Resorption by Shifting Macrophage-Osteoclast Polarization

(2014) Zixue Jin et al.   Cell Metabolism

NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease

(2014) Raffaele Cerutti et al.   Cell Metabolism

Reactive oxygen species and excitation–contraction coupling in the context of cardiac pathology

(2014) Anne C. Köhler et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Tri-iodo-l-thyronine promotes the maturation of human cardiomyocytes-derived from induced pluripotent stem cells

(2014) Xiulan Yang et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

A multicenter study on Leigh syndrome: disease course and predictors of survival

(2014) Kalliopi Sofou et al.   Orphanet Journal of Rare Diseases

Complex I Deficiency Due to Selective Loss of Ndufs4 in the Mouse Heart Results in Severe Hypertrophic Cardiomyopathy

(2014) Edward T. Chouchani et al.   PLoS One

Mitochondrial Complex I Deficiency Increases Protein Acetylation and Accelerates Heart Failure

(2013) Georgios Karamanlidis et al.   Cell Metabolism

Ex-527 inhibits Sirtuins by exploiting their unique NAD+-dependent deacetylation mechanism

(2013) M. Gertz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

(2013) S. C. Johnson et al.   SCIENCE

Silent Information Regulator 1 Protects the Brain Against Cerebral Ischemic Damage

(2013) M. Hernandez-Jimenez et al.   STROKE

Distinct Metabolic Flow Enables Large-Scale Purification of Mouse and Human Pluripotent Stem Cell-Derived Cardiomyocytes

(2012) Shugo Tohyama et al.   Cell Stem Cell

Assessment of mitochondrial respiratory chain enzymatic activities on tissues and cultured cells

(2012) Marco Spinazzi et al.   Nature Protocols

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

Reactive Oxygen Species Originating From Mitochondria Regulate the Cardiac Sodium Channel

(2010) Man Liu et al.   CIRCULATION RESEARCH

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The cardiac sodium channel displays differential distribution in the conduction system and transmural heterogeneity in the murine ventricular myocardium

(2009) C. A. Remme et al.   BASIC RESEARCH IN CARDIOLOGY

Multiple Inference and Gender Differences in the Effects of Early Intervention: A Reevaluation of the Abecedarian, Perry Preschool, and Early Training Projects

(2009) Michael L. Anderson   JOURNAL OF THE AMERICAN STATISTICAL ASSOCIATION

Leigh Syndrome: Clinical and Neuroimaging Follow-Up

(2009) Hsiu-Fen Lee et al.   PEDIATRIC NEUROLOGY

Acetylation Is Indispensable for p53 Activation

(2008) Yi Tang et al.   CELL

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism