Development of a method to evaluate the fatty acid oxidation impairment by infection using cultured cells and tandem mass spectrometry, in children
Project/Area Number |
24659498
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Research Category |
Grant-in-Aid for Challenging Exploratory Research
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Allocation Type | Multi-year Fund |
Research Field |
Pediatrics
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Research Institution | Shimane University |
Principal Investigator |
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Project Period (FY) |
2012-04-01 – 2015-03-31
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Project Status |
Completed (Fiscal Year 2014)
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Budget Amount *help |
¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Fiscal Year 2014: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2013: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
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Keywords | 脂肪酸酸化 / セレウリド / タンデムマス法 / in vitro probe assay / 急性脳症 / 解熱剤 / heat stress / 脂肪酸β酸化 / β酸化能 / 低体温療法 / 細菌毒素 / 小児薬物 / 発熱ストレス |
Outline of Final Research Achievements |
The mechanism of acute encephalopathy in young children is often unknown in many cases. Onset of acute encephalopathy is not infrequently similar to that of congenital fatty acid oxidation (FAO) disorders. Influence of bacterial toxins, antipyretic drugs, or heat stress by hyperpyrexia on FAO was determined by in vitro probe (IVP) assay using cultured fibroblasts, and tandem mass spectrometry, changing the circumstance of cell culture. The results were as follows: (i) cereulide, a toxin of Bacillus cereus, which occasionally causes acute encephalopathy in infants, disturbed FAO; (ii) heat stress possibly causes exacerbation of FAO in long-chain FAO disorders; (iii) antipyretics, salicylate (metabolites of Aspirin), and Diclofenac disturbed FAO in normal control cells, while acetaminophen did not. These findings may be consistent with clinical observation. The above findings will be of help for prevention of acute encephalopathy in children with infectious disease and/or hyperpyrexia.
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Report
(4 results)
Research Products
(57 results)
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[Journal Article] Clinical and genetic investigation of 17 Japanese patients with hyperekplexia2015
Author(s)
Mine J, Taketani T, Yoshida K, Yokochi F, Kobayashi J, Maruyama K, Nanishi E, Ono M, Yokoyama A, Arai H, Tamaura S, Suzuki Y, Otsubo S, Hayashi T, Kimura M, Kishi K, Yamaguchi S
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Journal Title
Developmental Medicine & Child Neurology
Volume: 57
Issue: 4
Pages: 372-377
DOI
Related Report
Peer Reviewed
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[Journal Article] Functional Analysis of iPSC-derived Myocytes from a Patient with Carnitine Palmitoyltransferase II Deficiency2014
Author(s)
Tetsuhiko Yasuno, Kenji Osafune, Hidetoshi Sakurai, Isao Asaka, Akihito Tanaka, Seiji Yamaguchi, Kenji Yamada, Hirofumi Hitomi, Sayaka Arai, Yuko Kurose, Yasuki Higaki, Mizuki Sudo, Souichi Ando, Hitoshi Nakashima, Takao Saito, Hidetoshi Kaneoka
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Journal Title
Biochem. and Biophys. Res. Commun
Volume: (in press)
Issue: 2
Pages: 175-181
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Expanded newborn mass screening with MS/MS and medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in Japan2013
Author(s)
Yamaguchi S, Purevusren J, Kobayashi H, Hasegawa Y, Mushimoto Y, Yamada K, Takahashi T, Furui M, Taketani T, Fukuda S, Fukao T, Shigematsu Y
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Journal Title
日本マス・スクリーニング学会誌
Volume: 23
Pages: 270-276
Related Report
Peer Reviewed
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[Presentation] Improvement of fatty acid oxidation capacity of cells from fatty acid oxidation defects at low temperature: evaluation by in vitro probe assay2014
Author(s)
Yamaguchi S, Liu L, Furui M, Yamada K, Taketani T, Shibata N, Kobayashi H, Hasegawa Y, Fukuda S
Organizer
Annual Symposium of the Society for the Study of Inborn Errors of Metabolism
Place of Presentation
Congress Innsbruck, Innsbruck, Austria
Year and Date
2014-09-02 – 2014-09-05
Related Report
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[Presentation] Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and newborn screening in Japan2013
Author(s)
Yamaguchi S, Purevsuren J, Hasegawa Y, Kobayashi H, Mushimoto Y, Yamada K, Takahashi T, Furui M, Fukao T, Shigematsu Y, Fukuda S
Organizer
2013 Joint Meeting of the Newborn Screening and Genetic Testing Symposium (NBS>S) and the International Society for Neonatal Screening (ISNS)
Place of Presentation
Atlanta Marriott Marquis, Atlanta, USA
Related Report
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[Presentation] Two Japanese cases of adult onset myopathic form of gulutaric acidemia type II2013
Author(s)
Yamaguchi S, Yamada K, Kobayashi H, Takahashi T, Hasegawa Y, Purevsuren J, Ohkubo T, Watanabe M, Tsunemi T, Ishii A, Takuma H, Tamaoka A, Shigematsu Y, Fukuda S
Organizer
12th International Congress of Inborn Errors of Metabolism
Place of Presentation
Palau de Congressos de Catalunya, Barcelona
Related Report
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[Presentation] The firse case of HSD10 disease (2-Methyl-3-Hydroxybutyryl-coA dehydrogenase deficiency) from Asia2013
Author(s)
Fukao T, Akida K, Goto M, Kuwayama N, Morita M, Hori T, Aoyama Y, Venkatesan R, Wierenga R, Hasegawa Y, Shigematsu Y, Ohtake A, Moriyama Y, Usuda N, Hsegawa Y
Organizer
The 3rd Asian Congress for Inherited Metabolic Diseases (ACIMD), The 55th Annual Meeting of the Japanese Society for Inherited Metabolic Diseases (JSIMD)
Place of Presentation
Tokyo Bay Maihama Hotel Club Resort, Chiba
Related Report
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