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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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,720,000 (Direct Cost: ¥4,400,000、Indirect Cost: ¥1,320,000)
Fiscal Year 2013: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Fiscal Year 2012: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2011: ¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
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| Keywords | 中性脂肪 / 中性脂肪蓄積心筋血管症 / 栄養 / 中鎖脂肪酸 / 動脈硬化 |
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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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Report
(4 results)
Research Products
(33 results)
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[Journal Article] Peripheral leukocyte anomaly detected with routine automated hematology analyzer sensitive to adipose triglyceride lipase deficiency manifesting neutral lipid storage disease with myopathy/triglyceride deposit cardiomyovasculopathy.2014
Author(s)
Suzuki A, Nagasaka H, Ochi Y, Kobayashi K, Nakamura H, Nakatani D, S Yamaguchi, Yamaki S, Wada A, Shirata Y, Hui SP, Toda T, Kuroda H, Chiba H, Hirano K.
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Journal Title
Mol Genet Metab Rep
Volume: 1
Pages: 249-253
DOI
Related Report
Peer Reviewed / Open Access
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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)
Issue: 5
Pages: 905-18
DOI
Related Report
Peer Reviewed
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[Journal Article] Genetic mutations in adipose triglyceride lipase and myocardial up-regulation of peroxisome proliferated activated receptor-γ in patients with triglyceride deposit cardiomyovasculopathy.2014
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
Issue: 2
Pages: 574-579
DOI
Related Report
Peer Reviewed
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