Search Results - "Vaz, Frédéric M."

Acylcarnitines: reflecting or inflicting insulin resistance? by Schooneman, Marieke G, Vaz, Frédéric M, Houten, Sander M, Soeters, Maarten R

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Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies by Wang, Gang, McCain, Megan L, Yang, Luhan, He, Aibin, Pasqualini, Francesco Silvio, Agarwal, Ashutosh, Yuan, Hongyan, Jiang, Dawei, Zhang, Donghui, Zangi, Lior, Geva, Judith, Roberts, Amy E, Ma, Qing, Ding, Jian, Chen, Jinghai, Wang, Da-Zhi, Li, Kai, Wang, Jiwu, Wanders, Ronald J A, Kulik, Wim, Vaz, Frédéric M, Laflamme, Michael A, Murry, Charles E, Chien, Kenneth R, Kelley, Richard I, Church, George M, Parker, Kevin Kit, Pu, William T

“…Cardiomyocytes generated from induced pluripotent cells hold great promise for understanding and treating heart disease. William Pu and his colleagues apply…”
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Sodium taurocholate cotransporting polypeptide (SLC10A1) deficiency: Conjugated hypercholanemia without a clear clinical phenotype by Vaz, Frédéric M., Paulusma, Coen C., Huidekoper, Hidde, de Ru, Minke, Lim, Cynthia, Koster, Janet, Ho‐Mok, Kam, Bootsma, Albert H., Groen, Albert K., Schaap, Frank G., Oude Elferink, Ronald P. J., Waterham, Hans R., Wanders, Ronald J.A.

“…The enterohepatic circulation of bile salts is an important physiological route to recycle bile salts and ensure intestinal absorption of dietary lipids. The…”
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Retrospective study of long-term outcomes of enzyme replacement therapy in Fabry disease: Analysis of prognostic factors by Arends, Maarten, Biegstraaten, Marieke, Hughes, Derralynn A, Mehta, Atul, Elliott, Perry M, Oder, Daniel, Watkinson, Oliver T, Vaz, Frédéric M, van Kuilenburg, André B P, Wanner, Christoph, Hollak, Carla E M

“…Despite enzyme replacement therapy, disease progression is observed in patients with Fabry disease. Identification of factors that predict disease progression…”
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Peroxisomes can oxidize medium‐ and long‐chain fatty acids through a pathway involving ABCD3 and HSD17B4 by Violante, Sara, Achetib, Nihad, Roermund, Carlo W. T., Hagen, Jacob, Dodatko, Tetyana, Vaz, Frédéric M., Waterham, Hans R., Chen, Hongjie, Baes, Myriam, Yu, Chunli, Argmann, Carmen A., Houten, Sander M.

“…ABSTRACT Peroxisomes are essential organelles for the specialized oxidation of a wide variety of fatty acids, but they are also able to degrade fatty acids…”
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Bactericidal activity of amphipathic cationic antimicrobial peptides involves altering the membrane fluidity when interacting with the phospholipid bilayer by Omardien, Soraya, Drijfhout, Jan W., Vaz, Frédéric M., Wenzel, Michaela, Hamoen, Leendert W., Zaat, Sebastian A.J., Brul, Stanley

“…Amphipathic cationic antimicrobial peptides (AMPs) TC19 and TC84, derived from the major AMPs of human blood platelets, thrombocidins, and Bactericidal Peptide…”
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Inherited disorders of complex lipid metabolism: A clinical review by Xiao, Changrui, Rossignol, Francis, Vaz, Frédéric M., Ferreira, Carlos R.

“…Over 80 human diseases have been attributed to defects in complex lipid metabolism. A majority of them have been reported recently in the setting of rapid…”
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Fatty Acid Oxidation in Peroxisomes: Enzymology, Metabolic Crosstalk with Other Organelles and Peroxisomal Disorders by Wanders, Ronald J A, Vaz, Frédéric M, Waterham, Hans R, Ferdinandusse, Sacha

“…Peroxisomes play a central role in metabolism as exemplified by the fact that many genetic disorders in humans have been identified through the years in which…”
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The murine retinal pigment epithelium requires peroxisomal β-oxidation to maintain lysosomal function and prevent dedifferentiation by Kocherlakota, Sai, Das, Yannick, Swinkels, Daniëlle, Vanmunster, Maarten, Callens, Manon, Vinckier, Stefan, Vaz, Frédéric M, Sinha, Debasish, Van Veldhoven, Paul P, Fransen, Marc, Baes, Myriam

“…Retinal pigment epithelium (RPE) cells have to phagocytose shed photoreceptor outer segments (POS) on a daily basis over the lifetime of an organism, but the…”
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Enzymology of the carnitine biosynthesis pathway by Strijbis, Karin, Vaz, Frédéric M., Distel, Ben

“…The water‐soluble zwitterion carnitine is an essential metabolite in eukaryotes required for fatty acid oxidation as it functions as a carrier during transfer…”
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Aging selectively dampens oscillation of lipid abundance in white and brown adipose tissue by Held, Ntsiki M., Buijink, M. Renate, Elfrink, Hyung L., Kooijman, Sander, Janssens, Georges E., Luyf, Angela C. M., Pras-Raves, Mia L., Vaz, Frédéric M., Michel, Stephan, Houtkooper, Riekelt H., van Weeghel, Michel

“…Lipid metabolism is under the control of the circadian system and circadian dysregulation has been linked to obesity and dyslipidemia. These factors and…”
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Pyruvate dehydrogenase complex plays a central role in brown adipocyte energy expenditure and fuel utilization during short-term beta-adrenergic activation by Held, Ntsiki M., Kuipers, Eline N., van Weeghel, Michel, van Klinken, Jan Bert, Denis, Simone W., Lombès, Marc, Wanders, Ronald J., Vaz, Frédéric M., Rensen, Patrick C. N., Verhoeven, Arthur J., Boon, Mariëtte R., Houtkooper, Riekelt H.

“…Activation of brown adipose tissue (BAT) contributes to total body energy expenditure through energy dissipation as heat. Activated BAT increases the clearance…”
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Cardiolipin provides an essential activating platform for caspase-8 on mitochondria by Gonzalvez, Francois, Schug, Zachary T, Houtkooper, Riekelt H, MacKenzie, Elaine D, Brooks, David G, Wanders, Ronald J.A, Petit, Patrice X, Vaz, Frédéric M, Gottlieb, Eyal

“…Cardiolipin is a mitochondria-specific phospholipid known to be intimately involved with apoptosis. However, the lack of appropriate cellular models to date…”
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Disorders of fatty acid homeostasis by Vaz, Frédéric M, Ferdinandusse, Sacha, Salomons, Gajja S, Wanders, Ronald J A

“…Humans derive fatty acids (FA) from exogenous dietary sources and/or endogenous synthesis from acetyl-CoA, although some FA are solely derived from exogenous…”
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C26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and Man by van de Beek, Malu-Clair, Dijkstra, Inge M E, van Lenthe, Henk, Ofman, Rob, Goldhaber-Pasillas, Dalia, Schauer, Nicolas, Schackmann, Martin, Engelen-Lee, Joo-Yeon, Vaz, Frédéric M, Kulik, Wim, Wanders, Ronald J A, Engelen, Marc, Kemp, Stephan

“…X-linked adrenoleukodystrophy (ALD), a progressive neurodegenerative disease, is caused by mutations in ABCD1 and characterized by very-long-chain fatty acids…”
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Cardiolipin deficiency affects respiratory chain function and organization in an induced pluripotent stem cell model of Barth syndrome by Dudek, Jan, Cheng, I-Fen, Balleininger, Martina, Vaz, Frédéric M, Streckfuss-Bömeke, Katrin, Hübscher, Daniela, Vukotic, Milena, Wanders, Ronald J A, Rehling, Peter, Guan, Kaomei

“…Barth syndrome (BTHS) patients carrying mutations in tafazzin (TAZ1), which is involved in the final maturation of cardiolipin, present with dilated…”
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Temporal Effects of Safflower Oil Diet-Based Linoleic Acid Supplementation on Barth Syndrome Cardiomyopathy by Zhu, Siting, Pang, Jing, Nguyen, Anh, Tan, Changming, Tso, Alexandria, Huynh, Tiana, Gu, Yusu, Gustafsson, Asa B, Vaz, Frédéric M, Evans, Sylvia M, Fang, Xi

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Integrative Analysis of the Inflammatory Bowel Disease Serum Metabolome Improves Our Understanding of Genetic Etiology and Points to Novel Putative Therapeutic Targets by Di'Narzo, Antonio F., Houten, Sander M., Kosoy, Roman, Huang, Ruiqi, Vaz, Frédéric M., Hou, Ruixue, Wei, Gabrielle, Wang, Wenhui, Comella, Phillip H., Dodatko, Tetyana, Rogatsky, Eduard, Stojmirovic, Aleksandar, Brodmerkel, Carrie, Perrigoue, Jacqueline, Hart, Amy, Curran, Mark, Friedman, Joshua R., Zhu, Jun, Agrawal, Manasi, Cho, Judy, Ungaro, Ryan, Dubinsky, Marla C., Sands, Bruce E., Suárez-Fariñas, Mayte, Schadt, Eric E., Colombel, Jean-Frédéric, Kasarskis, Andrew, Hao, Ke, Argmann, Carmen

“…Polygenic and environmental factors are underlying causes of inflammatory bowel disease (IBD). We hypothesized that integration of the genetic loci controlling…”
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Cardiac lipidomic profiles in mice undergo changes from fetus to adult by Chen, Ze'e, Pan, Zhixiang, Huang, Can, Zhu, Xiangbin, Li, Na, Huynh, Helen, Xu, Junjie, Huang, Lei, Vaz, Frédéric M., Liu, Jie, Han, Zhen, Ouyang, Kunfu

“…Lipids are essential cellular components with many important biological functions. Disturbed lipid biosynthesis and metabolism has been shown to cause cardiac…”
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Long-term efficacy and safety of elamipretide in patients with Barth syndrome: 168-week open-label extension results of TAZPOWER by Thompson, William R., Manuel, Ryan, Abbruscato, Anthony, Carr, Jim, Campbell, John, Hornby, Brittany, Vaz, Frédéric M., Vernon, Hilary J.

“…Evaluate long-term efficacy and safety of elamipretide during the open-label extension (OLE) of the TAZPOWER trial in individuals with Barth syndrome (BTHS)…”
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