{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T15:37:21Z","timestamp":1790177841979,"version":"4.1.0"},"reference-count":40,"publisher":"Oxford University Press (OUP)","issue":"10","funder":[{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,5,15]]},"DOI":"10.1093\/hmg\/ddx097","type":"journal-article","created":{"date-parts":[[2017,3,9]],"date-time":"2017-03-09T15:20:05Z","timestamp":1489072805000},"page":"1890-1899","source":"Crossref","is-referenced-by-count":26,"title":["Downregulation of pathways implicated in liver inflammation and tumorigenesis of glycogen storage disease type Ia mice receiving gene therapy"],"prefix":"10.1093","volume":"26","author":[{"given":"Goo-Young","family":"Kim","sequence":"first","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joon Hyun","family":"Kwon","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun-Ho","family":"Cho","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Zhang","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian C.","family":"Mansfield","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"},{"name":"Foundation Fighting Blindness, Columbia, MD 21046, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janice Y.","family":"Chou","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2017,3,13]]},"reference":[{"key":"key\n\t\t\t\t20180803083027_ddx097-B1","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nrendo.2010.189","article-title":"Glycogen storage disease type I and G6Pase-\u03b2 deficiency: etiology and therapy","volume":"6","author":"Chou","year":"2010","journal-title":"Nat. Rev. Endocrinol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B2","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 (Suppl 1)","author":"Rake","year":"2002","journal-title":"Eur. J. Pediatr"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B3","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1056\/NEJM197602192940805","article-title":"Continuous nocturnal intragastric feeding for management of type 1 glycogen-storage disease","volume":"294","author":"Greene","year":"1976","journal-title":"N. Engl. J. Med"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B4","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1056\/NEJM198401193100306","article-title":"Cornstarch therapy in type I glycogen-storage disease","volume":"310","author":"Chen","year":"1984","journal-title":"N. Engl. J. Med"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B5","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. Inherit. Metab. Dis"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B6","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":"key\n\t\t\t\t20180803083027_ddx097-B7","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.cytogfr.2015.07.010","article-title":"Mutations leading to constitutive active gp130\/JAK1\/STAT3 pathway","volume":"26","author":"Pilati","year":"2015","journal-title":"Cytokine Growth Factor Rev"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B8","doi-asserted-by":"crossref","first-page":"1719","DOI":"10.1002\/hep.25717","article-title":"Prevention of hepatocellular adenoma and correction of metabolic abnormalities in murine glycogen storage disease type Ia by gene therapy","volume":"56","author":"Lee","year":"2012","journal-title":"Hepatology"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B9","doi-asserted-by":"crossref","first-page":"5115","DOI":"10.1093\/hmg\/ddv230","article-title":"Mice expressing reduced levels of hepatic glucose-6-phosphatase-alpha activity do not develop age-related insulin resistance or obesity","volume":"24","author":"Kim","year":"2015","journal-title":"Hum. Mol. Genet"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B10","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"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B11","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.ymgmr.2015.03.001","article-title":"Minimal hepatic glucose-6-phosphatase-alpha activity required to sustain survival and prevent hepatocellular adenoma formation in murine glycogen storage disease type Ia","volume":"3","author":"Lee","year":"2015","journal-title":"Mol. Genet. Metab. Rep"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B12","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1016\/j.ymgme.2013.06.014","article-title":"The Upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia","volume":"110","author":"Lee","year":"2013","journal-title":"Mol. Genet. Metab"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B13","doi-asserted-by":"crossref","first-page":"E751","DOI":"10.1152\/ajpendo.00745.2009","article-title":"AMPK and SIRT1: a long-standing partnership?","volume":"298","author":"Ruderman","year":"2010","journal-title":"Am. J. Physiol. Endocrinol. Metab"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B14","first-page":"214","article-title":"Calorie restriction: is AMPK a key sensor and effector?","volume":"26","author":"Cant\u00f3","year":"2011","journal-title":"Physiol. (Bethesda)"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B15","doi-asserted-by":"crossref","first-page":"1895","DOI":"10.1101\/gad.17420111","article-title":"AMP-activated protein kinase: an energy sensor that regulates all aspects of cell function","volume":"25","author":"Hardie","year":"2011","journal-title":"Genes Dev"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B16","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.mce.2013.05.013","article-title":"Pharmacological activation of AMPK suppresses inflammatory response evoked by IL-6 signalling in mouse liver and in human hepatocytes","volume":"375","author":"Nerstedt","year":"2013","journal-title":"Mol. Cell Endocrinol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B17","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1038\/nrc2734","article-title":"STATs in cancer inflammation and immunity: a leading role for STAT3","volume":"9","author":"Yu","year":"2009","journal-title":"Nat. Rev. Cancer"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B18","doi-asserted-by":"crossref","first-page":"397","DOI":"10.3109\/10409238.2013.789479","article-title":"NAD(+) metabolism: a therapeutic target for age-related metabolic disease","volume":"48","author":"Mouchiroud","year":"2013","journal-title":"Crit. Rev. Biochem. Mol. Biol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B19","doi-asserted-by":"crossref","first-page":"2442","DOI":"10.1016\/j.bbadis.2015.08.017","article-title":"Sirtuin regulation in aging and injury","volume":"1852","author":"Poulose","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B20","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1038\/sj.emboj.7600244","article-title":"Modulation of NF-kappaB-dependent transcription and cell survival by the SIRT1 deacetylase","volume":"23","author":"Yeung","year":"2004","journal-title":"EMBO J"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B21","first-page":"5050","article-title":"SIRT1 counteracted the activation of STAT3 and NF-kappaB to repress the gastric cancer growth","volume":"7","author":"Lu","year":"2014","journal-title":"Int. J. Clin. Exp. Med"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B22","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1038\/cr.2010.183","article-title":"NF-\u03baB and STAT3\u2013key players in liver inflammation and cancer","volume":"21","author":"He","year":"2011","journal-title":"Cell Res"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B23","doi-asserted-by":"crossref","first-page":"254.","DOI":"10.1186\/1476-4598-13-254","article-title":"Role of SIRT1 in regulation of epithelial-to-mesenchymal transition in oral squamous cell carcinoma metastasis","volume":"13","author":"Chen","year":"2014","journal-title":"Mol. Cancer"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B24","doi-asserted-by":"crossref","first-page":"637308.","DOI":"10.1155\/2014\/637308","article-title":"E-cadherin and gastric cancer: cause, consequence, and applications","volume":"2014","author":"Liu","year":"2014","journal-title":"Biomed. Res. Int"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B25","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1139\/h2012-091","article-title":"Modulating fibroblast growth factor 21 in hyperphagic OLETF rats with daily exercise and caloric restriction","volume":"37","author":"Fletcher","year":"2012","journal-title":"Appl. Physiol. Nutr. Metab"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B26","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1146\/annurev-physiol-021115-105339","article-title":"Understanding the physiology of FGF21","volume":"78","author":"Fisher","year":"2016","journal-title":"Annu. Rev. Physiol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B27","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1053\/j.gastro.2013.10.059","article-title":"Hepatic SIRT1 attenuates hepatic steatosis and controls energy balance in mice by inducing fibroblast growth factor 21","volume":"146","author":"Li","year":"2014","journal-title":"Gastroenterology"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B28","doi-asserted-by":"crossref","first-page":"e55615.","DOI":"10.1371\/journal.pone.0055615","article-title":"betaKlotho suppresses tumor growth in hepatocellular carcinoma by regulating Akt\/GSK-3beta\/cyclin D1 signaling pathway","volume":"8","author":"Ye","year":"2013","journal-title":"PLoS One"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B29","doi-asserted-by":"crossref","first-page":"3032","DOI":"10.1007\/s10620-015-3711-z","article-title":"Long-term administration of fibroblast growth factor 21 prevents chemically-induced hepatocarcinogenesis in mice","volume":"60","author":"Xu","year":"2015","journal-title":"Dig. Dis. Sci"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B30","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1016\/j.tips.2015.05.001","article-title":"Nitric oxide in liver diseases","volume":"36","author":"Iwakiri","year":"2015","journal-title":"Trends. Pharmacol. Sci"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B31","first-page":"46","article-title":"Potential role of signal transducer and activator of transcription (STAT)3 signaling pathway in inflammation, survival, proliferation and invasion of hepatocellular carcinoma","volume":"1835","author":"Subramaniam","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B32","doi-asserted-by":"crossref","first-page":"56.","DOI":"10.3389\/fphys.2012.00056","article-title":"Functional role of monocytes and macrophages for the inflammatory response in acute liver injury","volume":"3","author":"Zimmermann","year":"2012","journal-title":"Front Physiol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B33","doi-asserted-by":"crossref","first-page":"7335","DOI":"10.1007\/s13277-014-2163-y","article-title":"Epithelial to mesenchymal transition inducing transcription factors and metastatic cancer","volume":"35","author":"Tania","year":"2014","journal-title":"Tumour. Biol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B34","doi-asserted-by":"crossref","first-page":"2202","DOI":"10.1172\/JCI23076","article-title":"Impaired negative feedback suppression of bile acid synthesis in mice lacking betaKlotho","volume":"115","author":"Ito","year":"2005","journal-title":"J. Clin. Invest"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B35","doi-asserted-by":"crossref","first-page":"74.","DOI":"10.1186\/1471-2164-15-74","article-title":"Comprehensive analysis of beta-catenin target genes in colorectal carcinoma cell lines with deregulated Wnt\/beta-catenin signaling","volume":"15","author":"Herbst","year":"2014","journal-title":"BMC Genom"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B36","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1016\/j.jhep.2013.07.001","article-title":"Wnt signaling and hepatocarcinogenesis: molecular targets for the development of innovative anticancer drugs","volume":"59","author":"Pez","year":"2013","journal-title":"J. Hepatol"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B37","doi-asserted-by":"crossref","first-page":"27628","DOI":"10.1074\/jbc.M805711200","article-title":"SIRT1 modulation of the acetylation status, cytosolic localization, and activity of LKB1. Possible role in AMP-activated protein kinase activation","volume":"283","author":"Lan","year":"2008","journal-title":"J. Biol. Chem"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B38","first-page":"194","article-title":"Targeting LKB1 signaling in cancer","volume":"1835","author":"Korsse","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B39","doi-asserted-by":"crossref","first-page":"E2772","DOI":"10.1073\/pnas.1303628110","article-title":"Ex-527 inhibits Sirtuins by exploiting their unique NAD+-dependent deacetylation mechanism","volume":"110","author":"Gertz","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"key\n\t\t\t\t20180803083027_ddx097-B40","doi-asserted-by":"crossref","first-page":"3084","DOI":"10.1172\/JCI64401","article-title":"Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease","volume":"123","author":"Zhou","year":"2013","journal-title":"J. Clin. Invest"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/26\/10\/1890\/25420526\/ddx097.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T10:54:06Z","timestamp":1568890446000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/26\/10\/1890\/3067488"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,3,13]]},"references-count":40,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2017,3,13]]},"published-print":{"date-parts":[[2017,5,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddx097","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2017,5,15]]},"published":{"date-parts":[[2017,3,13]]}}}