J
Jean Bastin
Researcher at Paris Descartes University
Publications - 51
Citations - 2969
Jean Bastin is an academic researcher from Paris Descartes University. The author has contributed to research in topics: Mitochondrion & Bezafibrate. The author has an hindex of 24, co-authored 50 publications receiving 2667 citations. Previous affiliations of Jean Bastin include Centre national de la recherche scientifique & French Institute of Health and Medical Research.
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Journal ArticleDOI
Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects.
Jean-Paul Bonnefont,Fatima Djouadi,Carina Prip-Buus,Stéphanie Gobin,Arnold Munnich,Jean Bastin +5 more
TL;DR: Carnitine palmitoyltransferase (CPT) deficiencies are common disorders of mitochondrial fatty acid oxidation and features of brain and kidney dysorganogenesis are frequently seen in the neonatal-onset CPT2 deficiency, almost always lethal during the first month of life.
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A gender-related defect in lipid metabolism and glucose homeostasis in peroxisome proliferator- activated receptor alpha- deficient mice.
Fatima Djouadi,Carla J. Weinheimer,Jeffrey E. Saffitz,Clovis Pitchford,Jean Bastin,Frank J. Gonzalez,Daniel P. Kelly +6 more
TL;DR: A pivotal role for PPARalpha in lipid and glucose homeostasis in vivo is demonstrated and estrogen signaling pathways in the regulation of cardiac and hepatic lipid metabolism are implicate.
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S6 Kinase Deletion Suppresses Muscle Growth Adaptations to Nutrient Availability by Activating AMP Kinase
Victor Aguilar,Victor Aguilar,Samira Alliouachene,Samira Alliouachene,Athanassia Sotiropoulos,Athanassia Sotiropoulos,Andrew K. Sobering,Andrew K. Sobering,Yoni Athea,Yoni Athea,Fatima Djouadi,Sylvain Miraux,Eric Thiaudière,Marc Foretz,Marc Foretz,Benoit Viollet,Benoit Viollet,Philippe Diolez,Jean Bastin,Paule Bénit,Pierre Rustin,David Carling,Marco Sandri,Renée Ventura-Clapier,Renée Ventura-Clapier,Mario Pende,Mario Pende +26 more
TL;DR: It is shown that S6K-deficient skeletal muscle cells have increased AMP and inorganic phosphate levels relative to ATP and phosphocreatine, causing AMP-activated protein kinase (AMPK) upregulation, which controls the energy state of the cell and the AMPK-dependent metabolic program.
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Activation of Peroxisome Proliferator-Activated Receptor Pathway Stimulates the Mitochondrial Respiratory Chain and Can Correct Deficiencies in Patients’ Cells Lacking Its Components
TL;DR: This study suggests a rationale for a possible correction of moderate RC disorders due to mutations in nuclear genes, using existing drugs, and brings new insights into the role of PPAR in the regulation of the mitochondrial RC in human cells.
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Current issues regarding treatment of mitochondrial fatty acid oxidation disorders.
Ute Spiekerkoetter,Jean Bastin,Melanie B. Gillingham,Andrew A. M. Morris,Frits A. Wijburg,Bridget Wilcken +5 more
TL;DR: Critical questions that are currently under debate in mitochondrial fatty acid oxidation (FAO) defects are discussed, including the use of bezafibrates in myopathic long-chain defects and whether triheptanoin is more effective than even-chain MCT.