2013 Fiscal Year Final Research Report
Development of dietary therapy for triglyceride deposit cardiomyovasculopathy
| Project/Area Number |
23617010
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Integrated Nutrition Science
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| Research Institution | Osaka University |
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Principal Investigator |
KEN-ICHI Hirano 大阪大学, 医学(系)研究科(研究院), 助教 (30332737)
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| Co-Investigator(Kenkyū-buntansha) |
INOUE Hiroyasu 奈良女子大学, 生活環境部, 教授 (40183743)
TAKENAKA Masaru 神戸女子大学, 家政学部, 教授 (20222101)
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| Project Period (FY) |
2011 – 2013
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| Keywords | 中性脂肪 / 中性脂肪蓄積心筋血管症 |
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| Research Abstract |
Triglyceride deposit cardiomyovasculopathy (TGCV) is a novel clinical entity in which TG accumulates in both myocardium and coronary arteries, leading to severe heart disease. A known cause of TGCV is genetic mutations in the adipose triglyceride lipase (ATGL), an essential molecule for intracellular hydrolysis of TG. In this study, we examined in vitro and in vivo effect of medium chain triglyceride on intracellular TG metabolism in ATGL-deficient fibroblasts and ATGL knockout mice, respectively. Based up these results, we developed a dietary therapy in which majority of natural fata was replaced by MCT. We investigated the effect of MCT diet on cardiac and skeletal muscle symptoms in ATGL-deficient patients. The diet was tolerable and possibly favorable effects were observed.
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Research Products
(19 results)
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[Journal Article] Selective evaluation of high density lipoprotein from mouse small intestines by an in situ perfusion technique2014
Author(s)
Yamaguchi S, Zhang B, Tomonaga T, Seino U, Kanagawa A, Nagasaka H, Suzuki A, Miida T, Yamada S, Sasaguri Y, Doi T, Saku K, Okazaki M, Tochino Y, Hirano K
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Journal Title
J Lipid Res
Volume: 55:(5)
Pages: 905-918
DOI
Peer Reviewed
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[Journal Article] Genetic mutations in the adipose triglyceride lipase and myocardial up-regulation of peroxisome proliferated activated receptor-gamma in patients with triglyceride deposit cardiomyovasculopathy2014
Author(s)
Hirano K, Tanaka T, Ikeda Y, Yamaguchi S, Zaima N, Kobayashi K, Suzuki A, Sakata Y, Sakata Y, Kobayashi K, Toda T, Fukushima N, Ishibashi-Ueda H, Tavian D, Nagasaka H, Hui SP, Chiba H, Sawa Y, Hori M
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Journal Title
Biochem Biophys Res Commun
Volume: 443(Epub ahead of print)
Pages: 574-9
DOI
Peer Reviewed
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[Journal Article] Peripheral leucocytes'anomaly detected with routine automated hematology analyzer sensitive to adipose triglyceride lipase deficiency manifesting neutral lipid storage disease with myopathy/triglyceride deposit cardiomyovasculopathy
Author(s)
Suzuki A, Nagasaka H, Ochi Y, Kobayashi K, Nakamura H, Nakatani D, Yamaguchi S, Yamaki S, Wada A, Shirata Y, Hui SP, Toda T, Kuroda H, Chiba H, Hirano K
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Journal Title
DOI
Peer Reviewed
