Glucose Uptake and Triacylglycerol Synthesis Are Increased in Barth Syndrome Lymphoblasts

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• 作者：Edgard M. Mejia ; James C. Zinko ; Kristin D. Hauff ; Fred Y. Xu ; Amir Ravandi230
• 关键词：Cardiolipin ; Metabolism ; Glucose transport ; Triacylglycerol synthesis ; Barth syndrome
• 刊名：Lipids
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：52
• 期：2
• 页码：161-165
• 全文大小：
• 刊物类别：Chemistry and Materials Science
• 刊物主题：Lipidology; Neurochemistry; Medical Biochemistry; Nutrition; Medicinal Chemistry; Microbial Genetics and Genomics;
• 出版者：Springer Berlin Heidelberg
• ISSN：1558-9307
• 卷排序：52

文摘

Barth syndrome (BTHS) is an X-linked genetic disease resulting in loss of cardiolipin (Ptd₂Gro). Patients may be predisposed to hypoglycemia and exhibit increases in whole-body glucose disposal rates and a higher fat mass percentage. We examined the reasons for this in BTHS lymphoblasts. BTHS lymphoblasts exhibited a 60% increase (p < 0.004) in 2-[1,2-3H(N)]deoxy-d-glucose uptake, a 40% increase (p < 0.01) in glucose transporter-3 protein expression, an increase in phosphorylated-adenosine monophosphate kinase (AMPK) and a 58% increase (p < 0.001) in the phosphorylated-AMPK/AMPK ratio compared to controls. In addition, BTHS lymphoblasts exhibited a 90% (p < 0.001) increase in d-[U-14C]glucose incorporated into 1,2,3-triacyl-sn-glycerol (TAG) and a 29% increase (p < 0.025) in 1,2-diacyl-sn-glycerol acyltransferase-2 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-body glucose disposal rates, may be predisposed to hypoglycemia and exhibit a higher fat mass percentage.