{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T11:16:36Z","timestamp":1768821396569,"version":"3.49.0"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T00:00:00Z","timestamp":1484611200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T00:00:00Z","timestamp":1484611200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/100008794","name":"Research Manitoba","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008794","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000038","name":"Natural Sciences and Engineering Research Council of Canada","doi-asserted-by":"publisher","award":["RGPIN\/03640-2014"],"award-info":[{"award-number":["RGPIN\/03640-2014"]}],"id":[{"id":"10.13039\/501100000038","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002651","name":"Barth Syndrome Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002651","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Children's Hospital Research Institute of Manitoba"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Lipids"],"published-print":{"date-parts":[[2017,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Barth syndrome (BTHS) is an X\u2010linked genetic disease resulting in loss of cardiolipin (Ptd<jats:sub>2<\/jats:sub>Gro). Patients may be predisposed to hypoglycemia and exhibit increases in whole\u2010body glucose disposal rates and a higher fat mass percentage. We examined the reasons for this in BTHS lymphoblasts. BTHS lymphoblasts exhibited a 60% increase (<jats:italic>p<\/jats:italic> &lt; 0.004) in 2\u2010[1,2\u2010<jats:sup>3<\/jats:sup>H(<jats:italic>N<\/jats:italic>)]deoxy\u2010<jats:sc>d<\/jats:sc>\u2010glucose uptake, a 40% increase (<jats:italic>p<\/jats:italic> &lt; 0.01) in glucose transporter\u20103 protein expression, an increase in phosphorylated\u2010adenosine monophosphate kinase (AMPK) and a 58% increase (<jats:italic>p<\/jats:italic> &lt; 0.001) in the phosphorylated\u2010AMPK\/AMPK ratio compared to controls. In addition, BTHS lymphoblasts exhibited a 90% (<jats:italic>p<\/jats:italic> &lt; 0.001) increase in <jats:sc>d<\/jats:sc>\u2010[U\u2010<jats:sup>14<\/jats:sup>C]glucose incorporated into 1,2,3\u2010triacyl\u2010<jats:italic>sn<\/jats:italic>\u2010glycerol (TAG) and a 29% increase (<jats:italic>p<\/jats:italic> &lt; 0.025) in 1,2\u2010diacyl\u2010<jats:italic>sn<\/jats:italic>\u2010glycerol acyltransferase\u20102 activity compared to controls. Thus, BTHS lymphoblasts exhibit increased glucose transport and increased glucose utilization for TAG synthesis. These results may, in part, explain why BTHS patients exhibit an increase in whole\u2010body glucose disposal rates, may be predisposed to hypoglycemia and exhibit a higher fat mass percentage.<\/jats:p>","DOI":"10.1007\/s11745-017-4232-7","type":"journal-article","created":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T08:33:09Z","timestamp":1484641989000},"page":"161-165","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Glucose Uptake and Triacylglycerol Synthesis Are Increased in Barth Syndrome Lymphoblasts"],"prefix":"10.1002","volume":"52","author":[{"given":"Edgard M.","family":"Mejia","sequence":"first","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 753 McDermot Avenue Winnipeg MB R3E 0W3 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James C.","family":"Zinko","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 753 McDermot Avenue Winnipeg MB R3E 0W3 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kristin D.","family":"Hauff","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 753 McDermot Avenue Winnipeg MB R3E 0W3 Canada"},{"name":"Department of Pathology and Laboratory Medicine University of British Columbia Vancouver Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fred Y.","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 753 McDermot Avenue Winnipeg MB R3E 0W3 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amir","family":"Ravandi","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pathophysiology, DREAM Children's Hospital Research Institute of Manitoba University of Manitoba Winnipeg MB R3E 0T6 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grant M.","family":"Hatch","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 753 McDermot Avenue Winnipeg MB R3E 0W3 Canada"},{"name":"Biochemistry and Medical Genetics, Center for Research and Treatment of Atherosclerosis, DREAM Children's Hospital Research Institute of Manitoba University of Manitoba Winnipeg MB R3E 0T6 Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,1,17]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10863-015-9601-4"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2016.08.010"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-23"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2012.11.007"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-012-9486-x"},{"issue":"14","key":"e_1_2_6_7_1","first-page":"1","article-title":"Measurement of glucose uptake in cultured cells","volume":"12","author":"Yamamoto N","year":"2011","journal-title":"Curr Protoc Pharmacol"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.42.25810"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2015.12.017"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2014.01.055"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(76)90527-3"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1210\/endo.131.3.1505464"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00496.2009"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tvjl.2004.01.026"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00084.2013"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20040886"},{"key":"e_1_2_6_17_1","first-page":"1993","volume-title":"GeneReviews\u00ae","author":"Ferreira C","year":"2014"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M034728"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.2000.3952"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2014.03.007"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.13753"}],"container-title":["Lipids"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1007%2Fs11745-017-4232-7","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s11745-017-4232-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11745-017-4232-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/aocs.onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s11745-017-4232-7","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11745-017-4232-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T13:14:32Z","timestamp":1760966072000},"score":1,"resource":{"primary":{"URL":"https:\/\/aocs.onlinelibrary.wiley.com\/doi\/10.1007\/s11745-017-4232-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1,17]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2017,2]]}},"alternative-id":["10.1007\/s11745-017-4232-7"],"URL":"https:\/\/doi.org\/10.1007\/s11745-017-4232-7","archive":["Portico"],"relation":{},"ISSN":["0024-4201","1558-9307"],"issn-type":[{"value":"0024-4201","type":"print"},{"value":"1558-9307","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,1,17]]}}}