{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T18:13:25Z","timestamp":1776276805353,"version":"3.50.1"},"reference-count":43,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,12,9]],"date-time":"2020-12-09T00:00:00Z","timestamp":1607472000000},"content-version":"vor","delay-in-days":366,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/100007680","name":"NMRC","doi-asserted-by":"publisher","award":["NMRC\/CIRG\/1457\/2016"],"award-info":[{"award-number":["NMRC\/CIRG\/1457\/2016"]}],"id":[{"id":"10.13039\/100007680","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007680","name":"NMRC","doi-asserted-by":"publisher","award":["NRMC\/CSA\/0054\/2013"],"award-info":[{"award-number":["NRMC\/CSA\/0054\/2013"]}],"id":[{"id":"10.13039\/100007680","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["R01DK105434"],"award-info":[{"award-number":["R01DK105434"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,1,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Glycogen storage disease type Ia (GSD Ia) is caused by autosomal mutations in glucose-6-phosphatase \u03b1 catalytic subunit (G6PC) and can present with severe hypoglycemia, lactic acidosis and hypertriglyceridemia. In both children and adults with GSD Ia, there is over-accumulation of hepatic glycogen and triglycerides that can lead to steatohepatitis and a risk for hepatocellular adenoma or carcinoma. Here, we examined the effects of the commonly used peroxisomal proliferated activated receptor \u03b1 agonist, fenofibrate, on liver and kidney autophagy and lipid metabolism in 5-day-old G6pc \u2212\/\u2212 mice serving as a model of neonatal GSD Ia. Five-day administration of fenofibrate decreased the elevated hepatic and renal triglyceride and hepatic glycogen levels found in control G6pc \u2212\/\u2212 mice. Fenofibrate also induced autophagy and promoted \u03b2-oxidation of fatty acids and stimulated gene expression of acyl-CoA dehydrogenases in the liver. These findings show that fenofibrate can rapidly decrease hepatic glycogen and triglyceride levels and renal triglyceride levels in neonatal G6pc \u2212\/\u2212 mice. Moreover, since fenofibrate is an FDA-approved drug that has an excellent safety profile, our findings suggest that fenofibrate could be a potential pharmacological therapy for GSD Ia in neonatal and pediatric patients as well as for adults. These findings may also apply to non-alcoholic fatty liver disease, which shares similar pathological and metabolic changes with GSD Ia.<\/jats:p>","DOI":"10.1093\/hmg\/ddz290","type":"journal-article","created":{"date-parts":[[2019,12,3]],"date-time":"2019-12-03T12:12:18Z","timestamp":1575375138000},"page":"286-294","source":"Crossref","is-referenced-by-count":20,"title":["Fenofibrate rapidly decreases hepatic lipid and glycogen storage in neonatal mice with glycogen storage disease type Ia"],"prefix":"10.1093","volume":"29","author":[{"given":"Zollie A","family":"Yavarow","sequence":"first","affiliation":[{"name":"Department of Pharmacology, Duke University, Durham NC 27710, USA"},{"name":"Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham NC 27710, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hye-Ri","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham NC 27710, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lauren R","family":"Waskowicz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham NC 27710, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Boon-Huat","family":"Bay","sequence":"additional","affiliation":[{"name":"Department of Anatomy, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 117594, Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah P","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham NC 27710, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul M","family":"Yen","sequence":"additional","affiliation":[{"name":"Cardiovascular and Metabolic Disorders Program, Duke\u2014National University of Singapore Graduate Medical School Singapore, Singapore 169547, Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dwight D","family":"Koeberl","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics, Duke University Medical Center, Durham NC 27710, USA"},{"name":"Department of Molecular Genetics and Microbiology, Duke University, Durham, NC 27710, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2019,12,9]]},"reference":[{"key":"2020121511493046200_ref1","first-page":"497","article-title":"Liver and kidney in glycogen storage disease","volume":"82","author":"von Gierke","year":"1929","journal-title":"Beitr. 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