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To investigate the role of G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content> in human adipose\u2010derived mesenchymal stem cells (<jats:styled-content style=\"fixed-case\">hMSC<\/jats:styled-content>s), the <jats:italic>G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content><\/jats:italic> gene was mutated by <jats:styled-content style=\"fixed-case\">CRISPR<\/jats:styled-content>\/Cas9 technology and single cell\u2010derived <jats:italic>G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content><\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> <jats:styled-content style=\"fixed-case\">hMSC<\/jats:styled-content>s were established. <jats:italic>G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content><\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> <jats:styled-content style=\"fixed-case\">hMSC<\/jats:styled-content>s have significantly increased cell proliferation but impaired adipogenesis and osteogenesis. These phenotypes are associated with two mechanisms: i) metabolic reprogramming in <jats:italic>G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content><\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> <jats:styled-content style=\"fixed-case\">hMSC<\/jats:styled-content>s causing a metabolic shift toward glycolysis rather than oxidative phosphorylation and ii) increased cyclooxygenase\u20102\u2010derived prostaglandin E<jats:sub>2<\/jats:sub> secretion in <jats:italic>G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content><\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> <jats:styled-content style=\"fixed-case\">hMSC<\/jats:styled-content>s. This study demonstrates that G6<jats:styled-content style=\"fixed-case\">PT<\/jats:styled-content> is essential for proliferation and differentiation of <jats:styled-content style=\"fixed-case\">MSC<\/jats:styled-content>s, providing important insights into the <jats:styled-content style=\"fixed-case\">GSD<\/jats:styled-content>\u2010Ib phenotypes.<\/jats:p>","DOI":"10.1002\/1873-3468.12939","type":"journal-article","created":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T07:16:40Z","timestamp":1513235800000},"page":"162-171","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Aberrant proliferation and differentiation of glycogen storage disease type Ib mesenchymal stem cells"],"prefix":"10.1002","volume":"592","author":[{"given":"Sang Wan","family":"Sim","sequence":"first","affiliation":[{"name":"Department of Biotechnology and Bioinformatics College of Science and Technology Korea University Sejong 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