Michael Schlame

Biosketch / Results /

Michael Schlame

Associate Professor, Department of Anesthesiology, Perioperative Care, and Pain Medicine;Associate Professor, Department of Cell Biology
Anesthesiology, Perioperative Care, and Pain Medicine
Director Cardiac Anesthesia NYU Anesthesia Associates
NYU Critical Care Associates

Contact Info

560 First Avenue
New York, NY 10016


1985-1989 — Humboldt University - Berlin, Residency Training
2002-2004 — NYU School of Medicine, Residency Training

Research Summary

Barth syndrome is a hereditary cardiomyopathy that also affects skeletal muscles, growth, and neutrophils. The mutated gene (tafazzin) is homologous to a conserved family of phospholipid acyltransferases. Children with Barth syndrome are deficient in the mitochondrial phospholipid cardiolipin, suggesting that the primary defect of the disease may indeed be found in phospholipid metabolism and may specifically affect the phospholipids of mitochondria. We want to study the mechanism by which tafazzin mutation causes cardiomyopathy and skeletal muscle disease. First, we want to identify the enzymatic function of tafazzin. We will identify the intracellular localization of tafazzin, its impact on lipid composition, and its mechanism of action. Second, we want to examine the effect of tafazzin on structure and function of mitochondria. Since mitochondrial dysfunction is a plausible etiology of cardiomyopathy and skeletal muscle weakness, we will analyze mitochondrial ultrastructure and oxidative phosphorylation in cell lines with tafazzin deletion. Third, we want to explore a Drosophila model of Barth syndrome, which was created in our laboratory. We will study lipid metabolism, muscle physiology, morphology, and mitochondrial ultrastructure in fruit flies with tafazzin deletion. The Drosophila model will also be used for cardiac studies since flies contain a contractile fluid pumping organ that shares conserved features of cardiogenesis with all heart-forming creatures, including humans. The project will provide insight into the pathologic mechanism of a unique disease, which presents a novel pathway from lipid defect(s) to cardio-skeletal myopathy. Such information may be useful for the development of new therapeutic approaches to cardiomyopathy and skeletal muscle disease.

Research Interests

Cardiomyopathy, Lipid Biochemistry and Mitochondria.

Tafazzins from Drosophila and mammalian cells assemble in large protein complexes with a short half-life
Xu, Yang; Malhotra, Ashim; Claypool, Steven M; Ren, Mindong; Schlame, Michael
2015-01-26; 1567-7249,Mitochondrion - id: 1439892, year: 2015 JOURNAL ARTICLE

Membrane curvature modulation of protein activity determined by NMR
Epand, Richard M; D'Souza, Kenneth; Berno, Bob; Schlame, Michael
2014-05-22; 0006-3002,Biochimica & biophysica acta - id: 1003462, year: 2014 REVIEW

Metabolism and function of mitochondrial cardiolipin
Ren, Mindong; Phoon, Colin K L; Schlame, Michael
2014-04-30; 0163-7827,Progress in lipid research - id: 932362, year: 2014 REVIEW

The turnover of glycerol and acyl moieties of cardiolipin
Xu, Yang; Schlame, Michael
2014-03-10; 0009-3084,Chemistry & physics of lipids - id: 829212, year: 2014 Journal Article

Cardiolipin as key lipid of mitochondria in health and disease, Bari, Italy, September 17, 2013
Corcelli, Angela; Schlame, Michael
2014-01-23; 1438-7697,European journal of lipid science & technology : EJLST - id: 751592, year: 2013