3-Hydroxy-3-methylglutaryl coenzyme A lyase: targeting and processing in peroxisomes and mitochondria by Lyudmila I. Ashmarina, Alexey V. Pshezhetsky, Steven S. Branda, Grazia Isaya, Grant A. Mitchell

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Inborn Errors of Ketone Body Metabolism and Transport by Jörn Oliver Sass Dr. rer. nat., Toshiyuki Fukao MD, PhD, Grant A. Mitchell MD

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Insulin therapy in acute decompensation of holocarboxylase synthetase deficiency with hyperglycemia and ketoacidosis by Tanguy Demaret, Jean-Sébastien Joyal, Aspasia Karalis, Fabienne Parente, Marie-Ange Delrue, Grant A. Mitchell

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Adipose tissue deficiency of hormone-sensitive lipase causes fatty liver in mice. by Bo Xia, Guo He Cai, Hao Yang, Shu Pei Wang, Grant A Mitchell, Jiang Wei Wu

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Human hormone-sensitive lipase (HSL): expression in white fat corrects the white adipose phenotype of HSL-deficient mice by Mélanie Fortier, Krishnakant Soni, Nancy Laurin, Shu Pei Wang, Pascale Mauriège, Frank R. Jirik, Grant A. Mitchell

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Intermittent neurologic decompensation: An underrecognized presentation of tyrosine hydroxylase deficiency by Marjolaine Champagne, Gabriella A. Horvath, Sébastien Perreault, Julie Gauthier, Keith Hyland, Jean‐François Soucy, Grant A. Mitchell

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Atypical juvenile presentation of GM2 gangliosidosis AB in a patient compound-heterozygote for c.259G>T and c.164C>T mutations in the GM2A gene by Carla Martins, Catherine Brunel-Guitton, Anne Lortie, France Gauvin, Carlos R. Morales, Grant A. Mitchell, Alexey V. Pshezhetsky

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An Epistatic Interaction between <i>Pnpla2</i> and <i>Lipe</i> Reveals New Pathways of Adipose Tissue Lipolysis by Xiao Zhang, Cong Cong Zhang, Hao Yang, Krishnakant G. Soni, Shu Pei Wang, Grant A. Mitchell, Jiang Wei Wu

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Epistatic interaction between the lipase-encoding genes Pnpla2 and Lipe causes liposarcoma in mice. by Jiang Wei Wu, Christoph Preuss, Shu Pei Wang, Hao Yang, Bo Ji, Gregory W Carter, Rebecca Gladdy, Gregor Andelfinger, Grant A Mitchell

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Mildly elevated succinylacetone and normal liver function in compound heterozygotes with pathogenic and pseudodeficient FAH alleles by Hao Yang, Francis Rossignol, Denis Cyr, Rachel Laframboise, Shu Pei Wang, Jean-François Soucy, Marie-Thérèse Berthier, Yves Giguère, Paula J. Waters, Grant A. Mitchell

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A liver-specific defect of Acyl-CoA degradation produces hyperammonemia, hypoglycemia and a distinct hepatic Acyl-CoA pattern. by Nicolas Gauthier, Jiang Wei Wu, Shu Pei Wang, Pierre Allard, Orval A Mamer, Lawrence Sweetman, Ann B Moser, Lisa Kratz, Fernando Alvarez, Yves Robitaille, François Lépine, Grant A Mitchell

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Urolithin A exerts antiobesity effects through enhancing adipose tissue thermogenesis in mice. by Bo Xia, Xiao Chen Shi, Bao Cai Xie, Meng Qing Zhu, Yan Chen, Xin Yi Chu, Guo He Cai, Min Liu, Shi Zhen Yang, Grant A Mitchell, Wei Jun Pang, Jiang Wei Wu

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Deficiency of ASGR1 in pigs recapitulates reduced risk factor for cardiovascular disease in humans. by Baocai Xie, Xiaochen Shi, Yan Li, Bo Xia, Jia Zhou, Minjie Du, Xiangyang Xing, Liang Bai, Enqi Liu, Fernando Alvarez, Long Jin, Shaoping Deng, Grant A Mitchell, Dengke Pan, Mingzhou Li, Jiangwei Wu

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Triglyceride-derived fatty acids reduce autophagy in a model of retinal angiomatous proliferation by Emilie Heckel, Gael Cagnone, Tapan Agnihotri, Bertan Cakir, Ashim Das, Jin Sung Kim, Nicholas Kim, Geneviève Lavoie, Anu Situ, Sheetal Pundir, Ye Sun, Florian Wünnemann, Kerry A. Pierce, Courtney Dennis, Grant A. Mitchell, Sylvain Chemtob, Flavio A. Rezende, Gregor Andelfinger, Clary B. Clish, Philippe P. Roux, Przemyslaw Sapieha, Lois E.H. Smith, Jean-Sébastien Joyal

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