Role of calcium signaling on the endodermal stem cells in the metabolic syndrom from childhood.

• Project/Area Number: 23591504
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Kumamoto University
• Principal Investigator: NAKAMURA Kimitoshi 熊本大学, 医学部附属病院, 講師 (30336234)
• Project Period (FY): 2011 – 2013
• Project Status: Completed (Fiscal Year 2013)
• Budget Amount: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000); Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2011: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
• Keywords: メタボリック症候群 / 幹細胞 / 内胚葉 / カルシウム シグナル / メタボリックシンドローム / カルシウムシグナル / トランスジェニックマウス / 内胚葉系幹細胞 / 肝細胞 / )内胚葉系幹細胞 / 心筋症

Research Abstract

The metabolic syndrome is a high-risk group of arteriosclerotic diseases occurring such as obese, hyperlipidemia, hypertension and diabetes. It was elucidated that the onset of this metabolic syndrome was found in childhood recently. In this study, we established endodermal stem cells from calreticulin knockout mice and overexpression mice with tissue-specific calreticulin, and analyzed the function in the calcium signals. We elucidated a mechanism of the onset of the metabolic syndrome from the viewpoint of calcium signal which was important to the adjustment of the function of gene expression and the protein function.

Research Products

• [Journal Article] p.E66Q Mutation in the GLA Gene is Associated with a High Risk of Cerebral Small-Vessel Occlusion in Elderly Japanese Males (2014)
  • Author(s): Nakamura K, Sekijima Y, NakamuraK, Hattori K, Nagamatsu K, Shimizu Y, Yazaki M, Sakurai A, Endo F, Fukushima Y, Ikeda S
  • Journal Title: Eur J Neurol Volume: 21 Issue: 1 Pages: 49-56
  • DOI: 10.1111/ene.12214
  • Peer Reviewed
• [Journal Article] Current status of hepatic glycogen storage disease in Japan: clinical manifestations, treatments and long-term outcomes. (2013)
  • Author(s): Kido J, Nakamura K, Matsumoto S, Mitsubuchi H, Ohura T, Shigematsu Y, Yorifuji T, Kasahara M, Horikawa R and Endo F
  • Journal Title: J. Hum. Genet. Volume: 58 Issue: 5 Pages: 285-292
  • DOI: 10.1038/jhg.2013.17
  • NAID: 10031177224
  • Peer Reviewed
• [Journal Article] Nickel BE, Light PE, Dyck JRB, Lopaschuk GD, Kardami E, Opas M, and Michalak M Calreticulin induces dilated cardiomyopathy (2013)
  • Author(s): Lee D, Oka T, Hunter B, Robinson A, Papp S, Nakamura K, Srisakuldee W
  • Journal Title: Plos One Volume: 8
  • Peer Reviewed
• [Journal Article] VLCAD deficiency in a patient who recovered from VF, but died suddenly of an RSV infection (2013)
  • Author(s): Yamamoto A, Nakamura K, Matsumoto S, Iwai M, Shigematsu Y, Tajima G, Tsumura M, Okada S, Mitsubuchi H, Endo F
  • Journal Title: Pediatr Int Volume: 55
  • Peer Reviewed
• [Journal Article] Newborn screening for Fabry disease in Japan: Prevalence and genotypes of Fabry disease in a pilot study. (2013)
  • Author(s): Inoue T, Hattori K, Ihara K, Ishii A, Nakamura K, Hirose S
  • Journal Title: J. Hum. Genet. Volume: 58 Issue: 8 Pages: 548-52
  • DOI: 10.1038/jhg.2013.48
  • NAID: 10031195285
  • Peer Reviewed
• [Journal Article] Interactive network analysis of the plasma amino acids profile in a mouse model of hyperglycemia (2013)
  • Author(s): Tanaka T, Mochida T, Maki Y, Shiraki Y, Mori H, Matsumoto S, Shimbo K, Ando T, Nakamura K, Endo F, Okamoto M
  • Journal Title: Springerplus Volume: 2 Pages: 287-287
  • Peer Reviewed
• [Journal Article] Clinical features and management of organic acidemias in Japan. (2013)
  • Author(s): Fujisawa D, Nakamura K, Mitsubuchi H, Ohura T, Shigematsu Y, Yorifuji T, Kasahara M, Horikawa R, Endo F.
  • Journal Title: J Hum Genet Volume: 97 Issue: 12 Pages: 769-742
  • DOI: 10.1038/jhg.2013.97
  • Peer Reviewed
• [Journal Article] Interactive network analysis of the plasma amino acids profile in a mouse model of hyperglycemia. (2013)
  • Author(s): Tanaka T, Mochida T, Maki Y, Shiraki Y, Mori H, Matsumoto S, Shimbo K, Ando T, Nakamura K, Endo F, Okamoto M.
  • Journal Title: Springerplus. Volume: 2 Pages: 287-287
  • Peer Reviewed
• [Journal Article] VLCAD deficiency in a patient who recovered from VF, but died suddenly of an RSV infection. (2013)
  • Author(s): Yamamoto A, Nakamura K, Matsumoto S, Iwai M, Shigematsu Y, Tajima G, Tsumura M, Okada S, Mitsubuchi H, Endo F
  • Journal Title: Pediatr Int. Volume: 55 Pages: 775-778
  • Peer Reviewed
• [Journal Article] Calreticulin induces dilated cardiomyopathy. (2013)
  • Author(s): Lee D, Oka T, Hunter B, Robinson A, Papp S, Nakamura K, Srisakuldee W, Nickel BE, Light PE, Dyck JRB, Lopaschuk GD, Kardami E, Opas M, and Michalak M
  • Journal Title: Plos One Volume: 8
  • Peer Reviewed
• [Journal Article] VLCAD deficiency in a patient who recovered from VF, but died suddenly of an RSV infection. (2013)
  • Author(s): Yamamoto A, Nakamura K, Matsumoto S, Iwai M, Shigematsu Y, Tajima G, Tsumura M, Okada S, Mitsubuchi H, Endo F.
  • Journal Title: Pediatr Int. Volume: in press
  • Peer Reviewed
• [Journal Article] p.E66Q Mutation in the GLA Gene is Associated with a High Risk of Cerebral Small-Vessel Occlusion in Elderly Japanese Males. (2013)
  • Author(s): Nakamura K, Sekijima Y, NakamuraK, Hattori K, Nagamatsu K, Shimizu Y, Yazaki M, Sakurai A, Endo F, Fukushima Y, Ikeda S
  • Journal Title: Eur J Neurol Volume: in press Issue: 2 Pages: 349-352
  • DOI: 10.1111/ene.12151
  • Peer Reviewed
• [Journal Article] Identification of a novel mutation and prevalence study for fabry disease in Japanese dialysis patients. (2012)
  • Author(s): Nishino T, Obata Y, Furusu A, Hirose M, Shinzato K, Hattori K, Nakamura K, Matsumoto T, Endo F, Kohno S
  • Journal Title: Ren Fail. Volume: 34 Pages: 566-570
  • Peer Reviewed
• [Journal Article] Effect of body mass index-z score on adverse levels of cardiovascular disease risk factors (2012)
  • Author(s): Katsuren K, Nakamura K, Ohta T
  • Journal Title: Pediatr Int. Volume: 54 Pages: 200-204
  • Peer Reviewed
• [Journal Article] Newborn Screening for lysosomal disorders (2011)
  • Author(s): Nakamura K, Hattori K, Endo F.
  • Journal Title: Am J Med Genet Volume: 157 Pages: 63-71
• [Journal Article] Improved assay for differential diagnosis between Pompe disease and acid alpha-glucosidase pseudodeficiency on dried blood spots (2011)
  • Author(s): Shigeto S, Katafuchi T, Okada Y, Nakamura K, Endo F, Okuyama T, Takeuchi H, Kroos MA, Verheijen FW, Reuser AJJ, Okumiya T
  • Journal Title: Mol. Genet. Metab Volume: 103 Pages: 12-17
• [Journal Article] Time-dependent changes in the plasma amino acid concentration in diabetes mellitus (2011)
  • Author(s): Mochida T, Tanaka T, Shiraki Y, Tajiri H, Matsumoto S, Shimbo K, Ando T, Nakamura K, Okamoto M, Endo F. . , ()
  • Journal Title: Mol Genet Metab Volume: 103 Pages: 406-409
• [Journal Article] Long-term outcome and intervention of urea cycle disorders in Japan (2011)
  • Author(s): Kido J, Nakamura K, Mitsubuchi H, Ohura T, Takayanagi M, Matsuo M, Yoshino M, Shigematsu Y, Yorifuji T, Kasahara M, Horikawa R and Endo F
  • Journal Title: Journal of Inherited Metabolic Disease Volume: 10.1007
• [Journal Article] 高アンモニア血症、血中シトルリン高値、BUN高値 日本先天代謝異常学会 編 (2011)
  • Author(s): 中村公俊 他
  • Journal Title: 先天代謝異常症Diagnosis at a Glance Volume: 初版 Pages: 124-126
• [Presentation] Urea Cycle Disorders in Japan (2013)
  • Author(s): Kimitoshi Nakamura
  • Organizer: SHARE Meeting 2013
  • Place of Presentation: Mövenpick Hotel Amsterdam City Centre, Amsterdam, Netherlands
  • Year and Date: 2013-06-27
• [Presentation] Urea Cycle Amino Acids,Properties and Clinical Application. Role of Citrulline and Urea Cycle Disorders (2013)
  • Author(s): Kimitoshi Nakamura
  • Organizer: The 8th Asia Pacific Conference on Clinical Nutrition, 2013 The Tokyo Bay Maihama Hotel Club
  • Place of Presentation: Tokyo, Japan
  • Year and Date: 2013-06-10
• [Presentation] Screening for Fabry Disease in Japan (2013)
  • Author(s): Kimitoshi Nakamura, MD
  • Organizer: 2013 Joint Meeting of the Newborn Screening and Genetic Testing Symposium and the International Society for Neonatal Screening
  • Place of Presentation: Atlanta Marriott Marquis(Atlanta, GA,USA)
• [Presentation] Screening for Fabry Disease in Japan (2013)
  • Author(s): Kimitoshi Nakamura
  • Organizer: International Society for Neonatal Screening
  • Place of Presentation: Atranta USA
• [Presentation] Newborn Screening in Japan. (2012)
  • Author(s): Nakamura K
  • Organizer: The 10th Asia-Pacific Conference on Human Genetics
  • Place of Presentation: Crowne Plaza, Kuala Lumpur, Malaysia
• [Presentation] Screening for Fabry Disease in Japan (2011)
  • Author(s): K.Nakamura, K.Hattori, S.Matsumoto, H.Mitsubuchi, F.Endo
  • Organizer: 12thICHG Montreal2011
  • Place of Presentation: Montreal Canada
• [Presentation] ろ紙血を用いたファブリー病のスクリーニング (2011)
  • Author(s): 中村公俊，服部希世子，田崎隆二，松本志郎，三渕浩，遠藤文夫
  • Organizer: 第３８回日本マス・スクリーニング学会
  • Place of Presentation: 福井県県民ホール（福井）
• [Presentation] ろ紙血を用いたファブリー病のスクリーニングとαガラクトシダーゼ遺伝子のE66Q変異 (2011)
  • Author(s): 中村公俊，服部希世子，松本志郎，三渕浩，遠藤文夫
  • Organizer: 第56回人類遺伝学会
  • Place of Presentation: 幕張メッセ（千葉）
• [Presentation] 尿素サイクル異常症（OTC欠損症、CPS1欠損症）とリジン尿性蛋白不耐症におけるシトルリン治療 (2011)
  • Author(s): 中村公俊 他
  • Organizer: 第53回日本先天代謝異常学会
  • Place of Presentation: ホテルニューオータニ幕張（千葉）
• [Presentation] 尿素サイクル異常症（UCD）の治療法‐風はどっちにふいている？‐ (2011)
  • Author(s): 中村公俊
  • Organizer: 第53回日本先天代謝異常学会
  • Place of Presentation: ホテルニューオータニ幕張（千葉）
• [Presentation] ろ紙血を用いたファブリー病のスクリーニング (2011)
  • Author(s): 中村公俊，服部希世子，松本志郎，田崎隆二，三渕浩，遠藤文夫
  • Organizer: 第53回日本先天代謝異常学会
  • Place of Presentation: ホテルニューオータニ幕張（千葉）
• [Presentation] UREA CYCLE AMINO ACIDS; ARGININE, ORNITHINE AND CITRULLINE- PROPERTIES AND CLINICAL APPLICATION
  • Author(s): Kimitoshi Nakamura
  • Organizer: The 8th Asia Pacific Conference on Clinical Nutrition
  • Place of Presentation: Tokyo Bay Maihama Hotel Club Resort, Tokyo, Japan
  • Invited
• [Presentation] Screening for Fabry Disease in Japan.
  • Author(s): Nakamura K
  • Organizer: 2012 Joint Conference of Medical Genetics, Genomics & Korean LSD Symposium.
  • Place of Presentation: Asan Medical Center, Seoul, Korea
  • Invited
• [Presentation] Overview of Japan Newborn Screening Experience for Pompe Disease
  • Author(s): Nakamura K
  • Organizer: 14th Asia LSD Symposium
  • Place of Presentation: Grand Hyatt Beijing Chaina
  • Invited
• [Presentation] Screening for Fabry Disease in Japan.
  • Author(s): Nakamura K
  • Organizer: 4th International Forum for Lysosomal Storage Disease
  • Place of Presentation: Jikei University Auditorium, Tokyo
• [Book] 尿素サイクル異常症 小児疾患の診断治療基準 第4版 小児内科 第44巻増刊号 (2012)
  • Author(s): 中村公俊
  • Total Pages: 1132
  • Publisher: 東京医学社 (2012)