T
Teresa C. Leone
Researcher at University of Pennsylvania
Publications - 53
Citations - 9092
Teresa C. Leone is an academic researcher from University of Pennsylvania. The author has contributed to research in topics: Peroxisome proliferator-activated receptor & Beta oxidation. The author has an hindex of 36, co-authored 51 publications receiving 8183 citations. Previous affiliations of Teresa C. Leone include University of Utah & Washington University in St. Louis.
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Journal ArticleDOI
A critical role for the peroxisome proliferator-activated receptor alpha (PPARalpha) in the cellular fasting response: the PPARalpha-null mouse as a model of fatty acid oxidation disorders.
TL;DR: A critical role for PPAR alpha is defined in a transcriptional regulatory response to fasting and the PPARalpha-/- mouse is identified as a potentially useful murine model of inborn and acquired abnormalities of human fatty acid utilization.
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PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis.
Teresa C. Leone,John J. Lehman,Brian N. Finck,Paul J. Schaeffer,Adam R. Wende,Sihem Boudina,Michael Courtois,David F. Wozniak,Nandakumar Sambandam,Carlos Bernal-Mizrachi,Zhouji Chen,John O. Holloszy,Denis M. Medeiros,Robert E. Schmidt,Jeffrey E. Saffitz,E. Dale Abel,Clay F. Semenkovich,Daniel P. Kelly +17 more
TL;DR: It is demonstrated that PGC-1α is necessary for appropriate adaptation to the metabolic and physiologic stressors of postnatal life.
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The cardiac phenotype induced by PPARα overexpression mimics that caused by diabetes mellitus
Brian N. Finck,John J. Lehman,Teresa C. Leone,Michael J. Welch,Michael J. Bennett,Attila Kovacs,Xianlin Han,Richard W. Gross,Ray Kozak,Gary D. Lopaschuk,Daniel P. Kelly +10 more
TL;DR: PPARalpha is a critical regulator of myocardial fatty acid uptake and utilization, activation of cardiac PPARalpha regulatory pathways results in a reciprocal repression of glucose uptake and usage pathways, and derangements in myocardian energy metabolism typical of the diabetic heart can become maladaptive, leading to cardiomyopathy.
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Lipin 1 is an inducible amplifier of the hepatic PGC-1α/PPARα regulatory pathway
Brian N. Finck,Matthew C. Gropler,Zhouji Chen,Teresa C. Leone,Michelle A. Croce,Thurl E. Harris,John C. Lawrence,Daniel P. Kelly +7 more
TL;DR: It is demonstrated that the expression of lipin 1 is induced by peroxisome proliferator-activated receptor gamma (PPARgamma) coactivator 1alpha (PGC-1alpha), a transcriptional coactivators controlling several key hepatic metabolic pathways.
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Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs
Changsung Kim,Johnson Wong,Jianyan Wen,Shirong Wang,Cheng Wang,Sean Spiering,Natalia G. Kan,Sonia Vanina Forcales,Pier Lorenzo Puri,Teresa C. Leone,Joseph E. Marine,Hugh Calkins,Daniel P. Kelly,Daniel P. Judge,Huei Sheng Vincent Chen,Huei Sheng Vincent Chen +15 more
TL;DR: This study is the first to demonstrate that induction of adult-like metabolism has a critical role in establishing an adult-onset disease model using patient-specific iPSCs, and revealed crucial pathogenic insights that metabolic derangement in adult- like metabolic milieu underlies ARVD/C pathologies, enabling us to propose novel disease-modifying therapeutic strategies.