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Long-term complications of GSD Ia include hepatic adenomas and carcinomas, in association with the suppression of autophagy in the liver. The G6pc\u2212\/\u2212 mouse and canine models for GSD Ia were treated with the pan-peroxisomal proliferator-activated receptor agonist, bezafibrate, to determine the drug\u2019s effect on liver metabolism and function. Hepatic glycogen and triglyceride concentrations were measured and western blotting was performed to investigate pathways affected by the treatment. Bezafibrate decreased liver triglyceride and glycogen concentrations and partially reversed the autophagy defect previously demonstrated in GSD Ia models. Changes in medium-chain acyl-CoA dehydrogenase expression and acylcarnintine flux suggested that fatty acid oxidation was increased and fatty acid synthase expression associated with lipogenesis was decreased in G6pc\u2212\/\u2212 mice treated with bezafibrate. In summary, bezafibrate induced autophagy in the liver while increasing fatty acid oxidation and decreasing lipogenesis in G6pc\u2212\/\u2212 mice. It represents a potential therapy for glycogen overload and hepatosteatosis associated with GSD Ia, with beneficial effects that have implications for non-alcoholic fatty liver disease.<\/jats:p>","DOI":"10.1093\/hmg\/ddy343","type":"journal-article","created":{"date-parts":[[2018,9,24]],"date-time":"2018-09-24T15:12:59Z","timestamp":1537801979000},"page":"143-154","source":"Crossref","is-referenced-by-count":41,"title":["Bezafibrate induces autophagy and improves hepatic lipid metabolism in glycogen storage disease type Ia"],"prefix":"10.1093","volume":"28","author":[{"given":"Lauren R","family":"Waskowicz","sequence":"first","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Zhou","sequence":"additional","affiliation":[{"name":"Cardiovascular and Metabolic Disorders Program, Duke-NUS Graduate Medical School Singapore, Singapore, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dustin J","family":"Landau","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth D","family":"Brooks","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"},{"name":"Division of Laboratory Animal Resources, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Lim","sequence":"additional","affiliation":[{"name":"Cardiovascular and Metabolic Disorders Program, Duke-NUS Graduate Medical School Singapore, Singapore, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zollie A","family":"Yavarow","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsubasa","family":"Kudo","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Tohoku University, Sendai, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haoyue","family":"Zhang","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yajun","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Anatomy, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart","family":"Grant","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah P","family":"Young","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bay Boon","family":"Huat","sequence":"additional","affiliation":[{"name":"Department of Anatomy, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M","family":"Yen","sequence":"additional","affiliation":[{"name":"Cardiovascular and Metabolic Disorders Program, Duke-NUS Graduate Medical School Singapore, Singapore, Singapore"},{"name":"Sarah W. 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Genet."},{"key":"2019102302292519100_ref2","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1016\/j.jhep.2011.08.025","article-title":"The interaction of hepatic lipid and glucose metabolism in liver diseases","volume":"56","author":"Bechmann","year":"2012","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref3","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1186\/1750-1172-6-27","article-title":"Glucose-6-phosphatase deficiency","volume":"6","author":"Froissart","year":"2011","journal-title":"Orphanet J. Rare Dis."},{"key":"2019102302292519100_ref4","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1038\/gim.2014.128","article-title":"Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics","volume":"16","author":"Kishnani","year":"2014","journal-title":"Genet. Med."},{"key":"2019102302292519100_ref5","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/s10545-005-7500-2","article-title":"Hepatocellular carcinoma in glycogen storage disease type Ia: a case series","volume":"28","author":"Franco","year":"2005","journal-title":"J. Inher. Metab. Dis."},{"key":"2019102302292519100_ref6","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1111\/liv.13031","article-title":"The story of HCC in NAFLD: from epidemiology, across pathogenesis, to prevention and treatment","volume":"36","author":"Margini","year":"2016","journal-title":"Liver Internat."},{"key":"2019102302292519100_ref7","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/j.ymgme.2009.04.003","article-title":"Activation of glycolysis and apoptosis in glycogen storage disease type Ia","volume":"97","author":"Sun","year":"2009","journal-title":"Mol. Genet. Metab."},{"key":"2019102302292519100_ref8","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1007\/s10059-009-0126-8","article-title":"Cell death and stress signaling in glycogen storage disease type I","volume":"28","author":"Kim","year":"2009","journal-title":"Mol. Cells"},{"key":"2019102302292519100_ref9","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.jhep.2010.08.014","article-title":"Targeted deletion of liver glucose-6 phosphatase mimics glycogen storage disease type 1a including development of multiple adenomas","volume":"54","author":"Mutel","year":"2011","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref10","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1016\/j.jhep.2015.10.008","article-title":"Induction of autophagy improves hepatic lipid metabolism in glucose-6-phosphatase deficiency","volume":"64","author":"Farah","year":"2016","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref11","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1002\/path.2694","article-title":"Autophagy: assays and artifacts","volume":"221","author":"Barth","year":"2010","journal-title":"J. Pathol."},{"key":"2019102302292519100_ref12","doi-asserted-by":"crossref","first-page":"R29","DOI":"10.1016\/j.cub.2011.11.034","article-title":"Mechanisms of autophagosome biogenesis","volume":"22","author":"Rubinsztein","year":"2012","journal-title":"Curr. Biol."},{"key":"2019102302292519100_ref13","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1128\/MCB.06159-11","article-title":"Role of AMPK-mTOR-Ulk1\/2 in the regulation of autophagy: cross talk, shortcuts, and feedbacks","volume":"32","author":"Alers","year":"2012","journal-title":"Mol. Cell. Biol."},{"key":"2019102302292519100_ref14","doi-asserted-by":"crossref","first-page":"2428","DOI":"10.1172\/JCI60580","article-title":"Thyroid hormone stimulates hepatic lipid catabolism via activation of autophagy","volume":"122","author":"Sinha","year":"2012","journal-title":"J. Clin. Invest."},{"key":"2019102302292519100_ref15","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1038\/nature07976","article-title":"Autophagy regulates lipid metabolism","volume":"458","author":"Singh","year":"2009","journal-title":"Nature"},{"key":"2019102302292519100_ref16","doi-asserted-by":"crossref","first-page":"1416","DOI":"10.1021\/bi00808a016","article-title":"Studies of lysosomal alpha-glucosidase. II. 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Diabetol."},{"key":"2019102302292519100_ref19","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/nature13961","article-title":"Nutrient-sensing nuclear receptors coordinate autophagy","volume":"516","author":"Lee","year":"2014","journal-title":"Nature"},{"key":"2019102302292519100_ref20","doi-asserted-by":"crossref","first-page":"193","DOI":"10.6065\/apem.2016.21.4.193","article-title":"Transcriptional coordination of hepatic autophagy by nutrient-sensing nuclear receptor PPAR\u03b1 and FXR","volume":"21","author":"Lee","year":"2016","journal-title":"Ann. Pediatr. Endocrinol. Metab."},{"key":"2019102302292519100_ref21","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1016\/j.jhep.2014.10.039","article-title":"Molecular mechanism of PPAR\u03b1 action and its impact on lipid metabolism, inflammation and fibrosis in non-alcoholic fatty liver disease","volume":"62","author":"Pawlak","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref22","doi-asserted-by":"crossref","first-page":"e1397","DOI":"10.1038\/cddis.2014.361","article-title":"Peroxisome proliferator-activated receptor \u03b1 activation attenuates the inflammatory response to protect the liver from acute failure by promoting the autophagy pathway","volume":"5","author":"Jiao","year":"2014","journal-title":"Cell. Death Dis."},{"key":"2019102302292519100_ref23","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1042\/BJ20111451","article-title":"Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling","volume":"441","author":"Lee","year":"2012","journal-title":"Biochem. J."},{"key":"2019102302292519100_ref24","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nrendo.2010.189","article-title":"Glycogen storage disease type I and G6Pase-beta deficiency: etiology and therapy","volume":"6","author":"Chou","year":"2010","journal-title":"Nat. Rev. Endocrinol."},{"key":"2019102302292519100_ref25","doi-asserted-by":"crossref","first-page":"5720","DOI":"10.1093\/emboj\/19.21.5720","article-title":"LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing","volume":"19","author":"Kabeya","year":"2000","journal-title":"EMBO J."},{"key":"2019102302292519100_ref26","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.ejphar.2012.07.028","article-title":"Alpha-lipoic acid induces adipose triglyceride lipase expression and decreases intracellular lipid accumulation in HepG2 cells","volume":"692","author":"Kuo","year":"2012","journal-title":"Eur. J. Pharmacol."},{"key":"2019102302292519100_ref27","doi-asserted-by":"crossref","first-page":"44408","DOI":"10.1038\/srep44408","article-title":"Hepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa)","volume":"7","author":"Farah","year":"2017","journal-title":"Sci. Rep."},{"key":"2019102302292519100_ref28","doi-asserted-by":"crossref","first-page":"210","DOI":"10.3350\/cmh.2013.19.3.210","article-title":"Nonalcoholic fatty liver disease: molecular mechanisms for the hepatic steatosis","volume":"19","author":"Koo","year":"2013","journal-title":"Clin. Mol. Hepatol."},{"key":"2019102302292519100_ref29","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1203\/PDR.0b013e31816c9013","article-title":"Increased de novo lipogenesis and delayed conversion of large VLDL into intermediate density lipoprotein particles contribute to hyperlipidemia in glycogen storage disease type 1a","volume":"63","author":"Bandsma","year":"2008","journal-title":"Pediatr. Res."},{"key":"2019102302292519100_ref30","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1172\/JCI88893","article-title":"Nutrient-sensing nuclear receptors PPAR\u03b1 and FXR control liver energy balance","volume":"127","author":"Preidis","year":"2017","journal-title":"J. Clin. Invest."},{"key":"2019102302292519100_ref31","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1006819","article-title":"Downregulation of SIRT1 signaling underlies hepatic autophagy impairment in glycogen storage disease type Ia","volume":"13","author":"Cho","year":"2017","journal-title":"PLoS Genet."},{"key":"2019102302292519100_ref32","doi-asserted-by":"crossref","first-page":"2042","DOI":"10.1002\/hep.29389","article-title":"Hepatocytes contribute to residual glucose production in a mouse model for glycogen storage disease type Ia","volume":"66","author":"Hijmans","year":"2017","journal-title":"Hepatology"},{"key":"2019102302292519100_ref33","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1038\/sj.gt.3302774","article-title":"Early, sustained efficacy of adeno-associated virus vector-mediated gene therapy in glycogen storage disease type Ia","volume":"13","author":"Koeberl","year":"2006","journal-title":"Gene Ther."},{"key":"2019102302292519100_ref34","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1354\/vp.38-1-83","article-title":"Canine model and genomic structural organization of glycogen storage disease type Ia (GSD Ia)","volume":"38","author":"Kishnani","year":"2001","journal-title":"Vet. Pathol."},{"key":"2019102302292519100_ref35","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1089\/hum.2011.106","article-title":"Long-term efficacy following readministration of an adeno-associated virus vector in dogs with glycogen storage disease type Ia","volume":"23","author":"Demaster","year":"2012","journal-title":"Hum. Gene Ther."},{"key":"2019102302292519100_ref36","article-title":"Long-term complications of glycogen storage disease type Ia in the canine model treated with gene replacement therapy","author":"Brooks","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"2019102302292519100_ref37","doi-asserted-by":"crossref","first-page":"R434","DOI":"10.1152\/ajpregu.00123.2014","article-title":"Chronic rapamycin treatment causes diabetes in male mice","volume":"307","author":"Schindler","year":"2014","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"2019102302292519100_ref38","doi-asserted-by":"crossref","first-page":"70","DOI":"10.3109\/07357907.2014.1001893","article-title":"Incidence and risk of high-grade stomatitis with mTOR inhibitors in cancer patients","volume":"33","author":"Shameem","year":"2015","journal-title":"Cancer Invest."},{"key":"2019102302292519100_ref39","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1053\/j.gastro.2018.06.083","article-title":"Mitochondrial dysfunction and signaling in chronic liver diseases","volume":"155","author":"Mansouri","year":"2018","journal-title":"Gastroenterology"},{"key":"2019102302292519100_ref40","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1038\/mt.2008.15","article-title":"AAV vector-mediated reversal of hypoglycemia in canine and murine glycogen storage disease type Ia","volume":"16","author":"Koeberl","year":"2008","journal-title":"Mol. Ther."},{"key":"2019102302292519100_ref41","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.ymgme.2017.01.003","article-title":"Glycogen storage disease type Ia mice with less than 2% of normal hepatic glucose-6-phosphatase-\u03b1 activity restored are at risk of developing hepatic tumors","volume":"120","author":"Kim","year":"2017","journal-title":"Mol. Genet. Metab."},{"issue":"Suppl. 1","key":"2019102302292519100_ref42","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1007\/s00431-002-0999-4","article-title":"Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European Study on Glycogen Storage Disease type I (ESGSD I)","volume":"161","author":"Rake","year":"2002","journal-title":"Eur. J. Pediatr."},{"key":"2019102302292519100_ref43","doi-asserted-by":"crossref","first-page":"47","DOI":"10.4103\/2156-7514.163991","article-title":"Malignant transformation of hepatic adenoma in glycogen storage disease type-1a: report of an exceptional case diagnosed on surveillance imaging","volume":"5","author":"Baheti","year":"2015","journal-title":"J. Clin. Imaging Sci."},{"key":"2019102302292519100_ref44","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.ymgme.2007.10.134","article-title":"Hepatocellular adenoma and metabolic balance in patients with type Ia glycogen storage disease","volume":"93","author":"Di Rocco","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"2019102302292519100_ref45","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.jhep.2010.03.002","article-title":"An adult male patient with multiple adenomas and a hepatocellular carcinoma: mild glycogen storage disease type la","volume":"53","author":"Cassiman","year":"2010","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref46","doi-asserted-by":"crossref","first-page":"4781","DOI":"10.1093\/hmg\/ddp441","article-title":"Chromosomal and genetic alterations in human hepatocellular adenomas associated with type Ia glycogen storage disease","volume":"18","author":"Kishnani","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"2019102302292519100_ref47","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1016\/j.jhep.2012.09.030","article-title":"Molecular characterization of hepatocellular adenomas developed in patients with glycogen storage disease type I","volume":"58","author":"Calderaro","year":"2013","journal-title":"J. Hepatol."},{"key":"2019102302292519100_ref48","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/8904_2014_344","article-title":"Regression of hepatocellular adenomas with strict dietary therapy in patients with glycogen storage disease type I","volume":"18","author":"Beegle","year":"2015","journal-title":"JIMD Rep."},{"key":"2019102302292519100_ref49","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.1002\/hep.26667","article-title":"Caffeine stimulates hepatic lipid metabolism by the autophagy-lysosomal pathway in mice","volume":"59","author":"Sinha","year":"2014","journal-title":"Hepatology"},{"key":"2019102302292519100_ref50","doi-asserted-by":"crossref","DOI":"10.1016\/j.molmet.2018.07.006","article-title":"Intracellular lipids are an independent cause of liver injury and chronic kidney disease in non alcoholic\nfatty liver disease-like context","author":"Monteillet","year":"2018","journal-title":"Mol. Metab."},{"key":"2019102302292519100_ref51","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1053\/j.gastro.2010.09.038","article-title":"Prevalence of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis among a largely middle-aged population utilizing ultrasound and liver biopsy: a prospective study","volume":"140","author":"Williams","year":"2011","journal-title":"Gastroenterology"},{"key":"2019102302292519100_ref52","doi-asserted-by":"crossref","first-page":"14018","DOI":"10.1038\/mtm.2014.18","article-title":"Assessment of toxicity and biodistribution of recombinant AAV8 vector-mediated immunomodulatory gene therapy in mice with Pompe disease","volume":"1","author":"Wang","year":"2014","journal-title":"Mol. Ther. Methods Clin. Dev."},{"key":"2019102302292519100_ref53","doi-asserted-by":"crossref","first-page":"1149","DOI":"10.1038\/nprot.2013.055","article-title":"Imaging of neutral lipids by oil red O for analyzing the metabolic status in health and disease","volume":"8","author":"Mehlem","year":"2013","journal-title":"Nat. Protoc."},{"key":"2019102302292519100_ref54","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/978-1-61737-985-7_3","article-title":"Acylcarnitines: analysis in plasma and whole blood using tandem mass spectrometry","volume-title":"Metabolic Profiling: Methods and Protocols","author":"Millington","year":"2011"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/28\/1\/143\/30262167\/ddy343.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/28\/1\/143\/30262167\/ddy343.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,10,23]],"date-time":"2019-10-23T02:30:17Z","timestamp":1571797817000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/28\/1\/143\/5106899"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,25]]},"references-count":54,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2018,9,25]]},"published-print":{"date-parts":[[2019,1,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddy343","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2019,1,1]]},"published":{"date-parts":[[2018,9,25]]}}}