Development of a new therapy for Rett syndrome model iPS cells and mouse by recombinant Ghrelin

2013 Fiscal Year Final Research Report

• Project/Area Number: 24791094
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Pediatrics
• Research Institution: Kurume University
• Principal Investigator: HARA Munetsugu 久留米大学, 医学部, 助教 (30389283)
• Project Period (FY): 2012-04-01 – 2014-03-31
• Keywords: レット症候群 / グレリン

Research Abstract

We revealed the gene expressions of ghrelin and ghrelin receptor during the differentiation of embryonic stem cells (ESCs) into embryoid body formation. We examined the effect of treatment with ghrelin during ESCs differentiation between the MeCP2-null ESCs and control wild-type ESCs. We remain no identification of the distinction between two ESCs groups on the factors affected by the treatment with ghrelin during ESCs differentiation. On the other hand, we found the distinct gene expressions of differentiation factors between two ESCs groups. Herein, we focused on the analysis of the roles of MeCP2 in the tissue influenced by the differentiation factors.
Moreover, we measured the concentrations of plasma ghrelin, serum GH, serum IGF-1, and the anthropometries (weight, hight, BMI, and head circumference) to investigate the relationship between the symptoms (failure of growth, epilepsy, and intellectual disability) and growth factors in the patients with Rett syndrome.

Research Products

• [Journal Article] Relation between circulating levels of GH, IGF-1, ghrelin and somatic growth in Rett Syndrome (2014)
  • Author(s): Hara M, Nishi Y, Yamashita Y, Hirata R, Takahashi S, Nagamitsu S, Hosoda H, Kangawa K, Kojima M, Matsuishi T
  • Journal Title: Brain Dev Volume: (in press)
  • Peer Reviewed
• [Journal Article] serial 18FDG-PET study of a patient with SSPE who had good prognosis by combination therapy with interferon alpha and ribavirin (2014)
  • Author(s): Ohya, T, Yamashita, Y, Shibuya, I, Hara, M, Nagamitsu, S, Kaida, H, Kurata, S, Ishibashi, M, Matsuishi, T
  • Journal Title: Eur J Pediatr Neurol Volume: (in press)
  • Peer Reviewed
• [Journal Article] An adult patient with mucolipidosis III alpha/beta presenting with parkinsonism (2013)
  • Author(s): Hara M, Inokuchi T, Taniwaki T, Otomo T, Sakai N, Matsuishi T, Yoshino M
  • Journal Title: Brain Dev Volume: 35(5) Pages: 462-465
  • Peer Reviewed
• [Journal Article] Eyelid myoclonia with absences occurring during the clinical course of cryptogenic myoclonic epilepsy of early childhood (2012)
  • Author(s): Ohya T, Yamashita Y, Shibuya I, Hara M, Nagamitsu S, Matsuishi T
  • Journal Title: Eur J Paediatr Neurol Volume: 16(4) Pages: 399-401
  • Peer Reviewed
• [Journal Article] Ghrelin levels are reduced in Rett syndrome patients with eating difficulties (2011)
  • Author(s): Hara M, Nishi Y, Yamashita Y, Yoh J, Takahashi S, Nagamitsu S, Kakuma T, Hosoda H, Kangawa K, Kojima M, Matsuishi T
  • Journal Title: Int J Devl Neuroscience Volume: 29(8) Pages: 899-902
  • Peer Reviewed
• [Journal Article] A case of clinically mild encephalitis with a reversible splenial lesion (MERS) after mumps vaccination (2011)
  • Author(s): Hara M, Mizuochi T, Kawano G, Koike T, Shibuya I, Ohya T, Ohbu K, Nagai K, Nagamitsu S, Yamashita Y, Nakayama T, Matsuishi T
  • Journal Title: Brain Dev Volume: 33(10) Pages: 842-4
  • Peer Reviewed
• [Journal Article] Prefrontal brain function in children with anorexia nervosa : a near-infrared spectroscopy study (2011)
  • Author(s): Nagamitsu S, Araki Y, Ioji T, Yamashita F, Ozono S, Kouno M, Iizuka C, Hara M, Shibuya I, Ohya T, Yamashita Y, Tsuda A, Kakuma T, Matsuishi T
  • Journal Title: Brain Dev Volume: 33(1) Pages: 35-44
  • Peer Reviewed
• [Presentation] 周期性嘔吐症を発症した精神遅滞, 自閉症を有する奇形症候群の1 例 (2013)
  • Author(s): 齊木玲央, 原宗嗣, 今城透, 澁谷郁彦, 大矢崇志, 永光信一郎, 山下裕史朗, 松石豊次郎
  • Organizer: 第55回日本小児神経学会学術集会
  • Place of Presentation: 大分
  • Year and Date: 2013-05-31
• [Presentation] 生後発達に伴うマウス脳内でのグレリン含量の変動に関する検討 (2013)
  • Author(s): 平田留美子, 西芳寛, 御船弘治, 細田洋司, 寒川賢治, 児島将康, 田中永一郎, 原宗嗣, 山下裕史朗, 松石豊次郎
  • Organizer: 第116回日本小児科学会学術総会
  • Place of Presentation: 広島
  • Year and Date: 2013-04-19
• [Presentation] Plasma ghrelin and serum IGF-1 in patients with Rett syndrome and its relationship to the head growth (2012)
  • Author(s): Hara M, Nishi Y, Yoh J, Takahashi S, Yamashita Y, Hosoda H, Kangawa K, Kojima M, Matsuishi T
  • Organizer: The 12th International Child Neurology Congress and the 11th Asia and Oceanian Congress of Child Neurology 2012
  • Place of Presentation: Brisbane, Australia
  • Year and Date: 20120527-0601
• [Presentation] 無症候性先天性サイトメガロウイルス感染症4 例の臨床的検討 (2012)
  • Author(s): 澁谷郁彦, 大矢崇志, 後藤憲次, 原宗嗣, 永光信一郎, 山下裕史朗, 松石豊次郎
  • Organizer: 第115回日本小児科学学術集会
  • Place of Presentation: 福岡
  • Year and Date: 2012-04-21
• [Presentation] 脳葉切除を行った側頭葉てんかん症例における脳血流の検討 (2012)
  • Author(s): 大矢崇志, 澁谷郁彦, 原宗嗣, 永光信一郎, 山下裕史朗, 松石豊次郎
  • Organizer: 第115回日本小児科学学術集会
  • Place of Presentation: 福岡
  • Year and Date: 2012-04-20
• [Presentation] Plasma concentration of ghrelin in patients with Rett syndrome together with their relationship to clinical manifestations (2011)
  • Author(s): Nishi Y, Hara M, Matsuishi T
  • Organizer: 第84回日本内分泌学会総会
  • Year and Date: 20110421-23
• [Presentation] Mecp2 遺伝子変異をもつレット症候群患者の血漿中グレリン濃度の検討 (2011)
  • Author(s): 原 宗嗣, 西 芳寛, 山下裕史朗, 松石豊次郎
  • Organizer: 第53回日本小児神経学会総会
  • Year and Date: 2011-05-26