A study of Reye-like syndrome associated with emetic toxin of bacillus cereus

2012 Fiscal Year Final Research Report

• Project/Area Number: 23791176
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Pediatrics
• Research Institution: Shimane University
• Principal Investigator: KOBAYASHI Hironori 島根大学, 医学部, 助教 (70397868)
• Project Period (FY): 2011 – 2012
• Keywords: 脂肪酸代謝障害 / ミトコンドリア / セレウス菌 / セレウリド / タンデムマス / アシルカルニチン

Research Abstract

This study was aimed to investigate the effect on the fatty acid metabolism of the patients with Bacillus cereus infection by an acylcarnitine analysis using tandem mass spectrometry, and to investigate effect of cereulide, emetic toxin from B. cereus, on fatty acid metabolism using in vitro acylcarnitine probe (IVP) assay of cultured fibroblasts. The acylcarnitie-profile of two patients with B. cereus infection revealed to be non-specific elevation of long-chain acylcarnitines. The result of IVP assay revealed that cereulide could inhibit fatty acid metabolism profiles fatty acid metabolism, resulting the similar acylcarnitine-profile to glutaric aciduria type 2. In addition, cereulide impaired fatty acid oxidation in concentration -dependent, the profile of acylcarnitines of IVP assay suggested toxic effect of cereulide on fatty acid oxidation was actualized from concentration of 50-100 ng/ml.

Research Products

• [Journal Article] Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: Evaluation by in vitro probe acylcarnitine assay (2012)
  • Author(s): Yamaguchi S, Li H, Purevsuren J, Yamada K, Furui M, Takahashi T, Mushimoto Y, Kobayashi H, Hasegawa Y, Taketani T, Fukao T, Fukuda S
  • Journal Title: Molecular Genetics and Metabolism Volume: 107 Pages: 87-91
  • Peer Reviewed
• [Journal Article] Clinical and molecular aspects of Japanese children with medium chain acyl-CoA dehydrogenase deficiency (2012)
  • Author(s): Purevsuren J, Hasegawa Y, Fukuda S, Kobayashi H, Mushimoto Y, Yamada K, Takahashi T, Fukao T, Yamaguchi S
  • Journal Title: Molecular Genetics and Metabolism Volume: 107 Pages: 237-240
  • Peer Reviewed
• [Journal Article] Retrospective review of Japanese sudden unexpected death in infancy: The importance of metabolic autopsy and expanded newborn screening (2011)
  • Author(s): Yamamoto T, Tanaka H, Kobayashi H, Okamura K, Tanaka T, Emoto Y, Sugimoto K, Nakatome M, Sakai N, Kuroki H, Yamaguchi S, Matoba R
  • Journal Title: Molecular Genetics and Metabolism Volume: 102(4) Pages: 399-406
  • Peer Reviewed
• [Journal Article] A patient with mitochondrial trifunctional protein deficiency due to the mutations in the HADHB gene showed recurrent myalgia since early childhood and was diagnosed in adolescence (2011)
  • Author(s): Yagi M, Lee T, Awano H, Tsuji M, Tajima G, Kobayashi H, Hasegawa Y, Yamaguchi S, Takeshima Y, Matsuo M
  • Journal Title: Molecular Genetics and Metabolism Volume: 104(4) Pages: 556-559
  • Peer Reviewed
• [Presentation] 新生児発症型グルタル酸尿症 2 型男児例に対する Bezafibrate 治療の経験 (2012)
  • Author(s): 小林弘典, 和田泰格, 鈴木絵理, 山田健治, 高橋知男, プレブスレン ジャミヤン, 長谷川有紀, 長谷川行洋, 山口清次
  • Organizer: 第54回日本先天代謝異常学会
  • Place of Presentation: (岐阜市)じゅうろくプラザ
  • Year and Date: 20121115-17
• [Presentation] ピボキシル基を含む抗菌薬によりカルニチン欠乏症をきたした 24 例の臨床像 (2012)
  • Author(s): 小林弘典, 高橋知男, 山田健治, 長谷川有紀, 山口清次
  • Organizer: 第64回中国四国小児科学会
  • Place of Presentation: (香川)香川大学
  • Year and Date: 20121110-11
• [Presentation] 新生児発症型グルタル酸尿症 2 型に対するベザフィブラートの有効性の検討: In vitro probe acylcarnitine assay の結果との関連性 (2012)
  • Author(s): 山 田 健 治 , 小林弘典 , Jamiyan Purevsuren, 高橋知男, 長谷川有紀, 山口清次
  • Organizer: 第37回日本医用マススペクトル学会年会
  • Place of Presentation: (名古屋)愛知県産業労働センター
  • Year and Date: 20121025-26
• [Presentation] Acylcarnitine analysis by ESI-MS/MS with smaller amount of sample and rapid analytical time (2012)
  • Author(s): Kobayashi H, Mushimoto Y, Hasegawa Y, Yamada K, Purevsuren J, Takahashi T, Minohata T, Iida J, Yamaguchi S
  • Organizer: 19th International Mass Spectrometry Conference
  • Place of Presentation: Kyoto International Conference Center, Japan
  • Year and Date: 20120915-21
• [Presentation] Application of in-vitro probe acylcarnitine assay using tandem mass spectrometry for the evaluation of mitochondrial fatty acid oxidation (2012)
  • Author(s): Prevsuren J, Kobayashi H, Hasegawa Y, Yamada K, Takahashi T, Yamaguchi S
  • Organizer: 19th International Mass Spectrometry Conference
  • Place of Presentation: Kyoto International Conference Center, Japan
  • Year and Date: 20120915-21
• [Presentation] セレウス菌食中毒による急性脳症はミトコンドリアβ酸化障害を介して起こる (2012)
  • Author(s): 山口清次, Prevsuren J, 小林弘典, 長谷川有紀
  • Organizer: 第54回日本小児神経学会
  • Place of Presentation: (札幌)ロイトン札幌
  • Year and Date: 20120517-19
• [Presentation] Beneficial effect of bezafibrate on boy with the late-onset gluraric aciduria type 2 (2012)
  • Author(s): Kobayashi H, Yamada K, Prevsuren J, Mushimoto Y, Takahashi T, Hasegawa Y,Ito M, Yamaguchi S
  • Organizer: The 2nd Asian Congress for Inherited Metabolic Diseases & The 12th Asian-European Workshop on Inborn Errors of Metabolism & The 12th Korean Congress of Inherited Metabolic Disease
  • Place of Presentation: Seoul, Korea
  • Year and Date: 20120401-06
• [Presentation] Emetic toxin of bacillus cereus that associates with reye-like syndrome severely inhibits mitochondrial fatty acid oxidation (2011)
  • Author(s): Purevsuren J, Hasegawa Y, Kobayashi H, Mushimoto Y, Yamada K, Okamoto A, Ohta M, Yamaguchi S
  • Organizer: Annual Symposium of the Society for the Study of Inborn Errors of Matabolism
  • Place of Presentation: Geneva, Switzerland
  • Year and Date: 20110830-0902