Barth Syndrome: Connecting Cardiolipin to Cardiomyopathy

Mitochondrial Metabolism in Aging Heart

(2016) Edward J. Lesnefsky et al.   CIRCULATION RESEARCH

On the origin of 3-methylglutaconic acid in disorders of mitochondrial energy metabolism

(2016) Nikita Ikon et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Cardiolipin metabolism and its causal role in the etiology of the inherited cardiomyopathy Barth syndrome

(2015) Gerard J. Gaspard et al.   CHEMISTRY AND PHYSICS OF LIPIDS

Metabolic annotation of 2-ethylhydracrylic acid

(2015) Robert O. Ryan   CLINICA CHIMICA ACTA

Left ventricular non-compaction cardiomyopathy

(2015) Jeffrey A Towbin et al.   LANCET

The Role of Cardiolipin in Cardiovascular Health

(2015) Zheni Shen et al.   Biomed Research International

Cardiac Metabolic Pathways Affected in the Mouse Model of Barth Syndrome

(2015) Yan Huang et al.   PLoS One

Cardiolipin remodeling: a regulatory hub for modulating cardiolipin metabolism and function

(2014) Cunqi Ye et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Metabolic biology of 3-methylglutaconic acid-uria: a new perspective

(2014) Betty Su et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

(2014) Gang Wang et al.   NATURE MEDICINE

Barth syndrome: Cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation

(2013) François Gonzalvez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Dysfunctional cardiac mitochondrial bioenergetic, lipidomic, and signaling in a murine model of Barth syndrome

(2013) Michael A. Kiebish et al.   JOURNAL OF LIPID RESEARCH

Barth syndrome

(2013) Sarah LN Clarke et al.   Orphanet Journal of Rare Diseases

Seven functional classes of Barth syndrome mutation

(2012) Kevin Whited et al.   HUMAN MOLECULAR GENETICS

Cardiolipin-dependent Reconstitution of Respiratory Supercomplexes from PurifiedSaccharomyces cerevisiaeComplexes III and IV

(2012) Soledad Bazán et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Intrafamilial variability for novel TAZ gene mutation: Barth syndrome with dilated cardiomyopathy and heart failure in an infant and left ventricular noncompaction in his great-uncle

(2012) Diti Ronvelia et al.   MOLECULAR GENETICS AND METABOLISM

The physical state of lipid substrates provides transacylation specificity for tafazzin

(2012) Michael Schlame et al.   Nature Chemical Biology

Impaired cardiac reserve and severely diminished skeletal muscle O2 utilization mediate exercise intolerance in Barth syndrome

(2011) Carolyn T. Spencer et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Cardiolipin microdomains localize to negatively curved regions of Escherichia coli membranes

(2011) L. D. Renner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Specific metabolic rates of major organs and tissues across adulthood: evaluation by mechanistic model of resting energy expenditure

(2010) ZiMian Wang et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Cardiac and Skeletal Muscle Defects in a Mouse Model of Human Barth Syndrome

(2010) Devrim Acehan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Formation of molecular species of mitochondrial cardiolipin2. A mathematical model of pattern formation by phospholipid transacylation

(2009) Michael Schlame   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Formation of molecular species of mitochondrial cardiolipin. 1. A novel transacylation mechanism to shuttle fatty acids between sn-1 and sn-2 positions of multiple phospholipid species

(2009) Ashim Malhotra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Identification of the Human Mitochondrial Linoleoyl-coenzyme A Monolysocardiolipin Acyltransferase (MLCL AT-1)

(2009) William A. Taylor et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cardiolipin, the heart of mitochondrial metabolism

(2008) R. H. Houtkooper et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria

(2008) Devrim Acehan et al.   MITOCHONDRION