Clinical significance of WASP protein complex in nucleus.

• Project/Area Number: 20591242
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Tohoku University
• Principal Investigator: SASAHARA Yoji Tohoku University, 病院, 講師 (60372314)
• Project Period (FY): 2008 – 2010
• Project Status: Completed (Fiscal Year 2010)
• Budget Amount: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000); Fiscal Year 2010: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2009: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2008: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
• Keywords: WASP / WIP / 原発性免疫不全症 / Wiskott-Aldrich症候群 / シグナル伝達 / マイクロアレイ / 免疫学

Research Abstract

Constitutively activating WASP causes X-linked neutropenia and myelodysplastic syndrome. We have identified that WASP played a significant role in transcriptional regulation of myeloid cell differentiation in nucleus. We also clarified that WIP formed a complex with WASP and regulated WASP protein stability, leading us to understand molecular pathogenesis of new type of WAS.

Research Products

• [Journal Article] Vincristine-resistant Kasabach-Meritt phenomenon successfully treated with low-dose irradiation. (2011)
  • Author(s): Watanabe Y, Sasahara Y, Tsuchiya S, ほか
  • Journal Title: Int.J.Hematol. 93 Pages: 126-128
  • Peer Reviewed
• [Journal Article] Vincristine-resistant Kasabach-Meritt phenomenon successfully treated With low-dose irradiation. (2011)
  • Author(s): Watanabe Y, Sasahara Y, et al.
  • Journal Title: Int.J.Hematol. Volume: 93 Pages: 126-128
  • Peer Reviewed
• [Journal Article] 4度の造血幹細胞移植を施行した重症再生不良性貧血に1例における内分泌学的合併症 (2010)
  • Author(s): 管野潤子、笹原洋二、土屋滋、ほか
  • Journal Title: 日本小児血液学会雑誌 24(1) Pages: 47-51
  • Peer Reviewed
• [Journal Article] 4度の造血幹細胞移植を施行した重症再生不良性貧血に1例における内分泌学的合併症 (2010)
  • Author(s): 管野潤子、笹原洋二, 他
  • Journal Title: 日本小児血液学会雑誌 Volume: 24(1) Pages: 47-51
  • Peer Reviewed
• [Journal Article] Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia. (2009)
  • Author(s): Horino S, Sasahara Y, Tsuchiya S, ほか
  • Journal Title: Int.J.Hematol. 90 Pages: 483-485
  • NAID: 10026109509
  • Peer Reviewed
• [Journal Article] Identification of severe combined immunodeficiency by T-cell receptor excision circles quiantification using Guthrie cards. (2009)
  • Author(s): Morinishi Y, Sasahara Y, Nonoyama S, ほか
  • Journal Title: J.Pediatr. 155(6) Pages: 829-833
  • Peer Reviewed
• [Journal Article] Refractory chronic immune thrombocytopenic purpura in a chila with acute lymphoblastic leukemia. (2009)
  • Author(s): Horino S, Sasahara Y, et al.
  • Journal Title: Int.J.Hematol. 90 Pages: 483-485
  • Peer Reviewed
• [Journal Article] Identification of severe combined immunodeficiency by T-cell receptor excision circles quiantification using Guthrie cards. (2009)
  • Author(s): Morinishi Y, Sasahara Y, et al.
  • Journal Title: J.Pediatr. 155(6) Pages: 829-833
  • Peer Reviewed
• [Presentation] ITPとの鑑別に重要なWAS/XLTの分子病態におけるWIPの役割 (2010)
  • Author(s): 笹原洋二、土屋滋、他
  • Organizer: 第52回日本小児血液学会
  • Place of Presentation: 大阪
  • Year and Date: 2010-12-17
• [Presentation] Wiskott-Aldrich症候群の分子病態からみた感染症とWIPの役割 (2010)
  • Author(s): 笹原洋二
  • Organizer: 第42回日本小児感染症学会
  • Place of Presentation: 仙台
  • Year and Date: 2010-11-27
• [Presentation] Wiskott-Aldrich症候群の分子病態の多様性とWIP欠損症発見の試み (2010)
  • Author(s): 笹原洋二、土屋滋、他
  • Organizer: 第72回日本血液学会
  • Place of Presentation: 横浜
  • Year and Date: 2010-09-26
• [Presentation] 骨髄非破壊的前処置にて造血幹細胞移植を施行した原発性免疫不全症6症例の検討 (2010)
  • Author(s): 笹原洋二、土屋滋、他
  • Organizer: 第72 回日本血液学会
  • Place of Presentation: 横浜
  • Year and Date: 2010-09-25
• [Presentation] 骨髄非破壊的前処置にて造血幹細胞移植を施行した原発性免疫不全症6症例の検討 (2010)
  • Author(s): 笹原洋二、土屋滋, 他
  • Organizer: 第72回日本血液学会
  • Place of Presentation: 横浜
  • Year and Date: 2010-09-25
• [Presentation] 骨髄非破壊的前処置にて造血幹細胞移植を施行した原発性免疫不全症6症例の検討 (2010)
  • Author(s): 笹原洋二、土屋滋、他
  • Organizer: 第113回日本小児科学会学術集会
  • Place of Presentation: 盛岡
  • Year and Date: 2010-04-24
• [Presentation] 悪性リンパ腫を合併したWiskott-Aldrich症候群患者に対するRISTによる骨髄移植の経験 (2010)
  • Author(s): 力石健、笹原洋二、他
  • Organizer: 第32回日本造血細胞移植学会総会
  • Place of Presentation: 浜松
  • Year and Date: 2010-02-20
• [Presentation] Role of WIP in Wiskott-Aldrich syndrome (2010)
  • Author(s): Yoji Sasahara
  • Organizer: The 2^<nd> Symposium for PID in Asia
  • Place of Presentation: 千葉、かずさDNA研究所
  • Year and Date: 2010-02-04
• [Presentation] WIP is a chaperone for WASP (2008)
  • Author(s): Yoji Sasahara
  • Organizer: 50^<th> American Society of Hematology (第50回米国血液学会) Annual Meeting
  • Place of Presentation: サンフランシスコ(米国)
  • Year and Date: 2008-12-06
• [Presentation] Meettheex pert ; Waltzing with WASP (2008)
  • Author(s): 笹原洋二
  • Organizer: 第50回日本小児血液学会
  • Place of Presentation: 幕張
  • Year and Date: 2008-11-16
• [Presentation] Meet the expert ; Waltzing with WASP (2008)
  • Author(s): 笹原洋二
  • Organizer: 第50回日本小児血液学会
  • Place of Presentation: 幕張
  • Year and Date: 2008-11-16
• [Presentation] WASP異常症の多様性とその分子病態の解明に向けて (2008)
  • Author(s): 笹原洋二、土屋滋、他
  • Organizer: 第50回日本小児血液学会
  • Place of Presentation: 幕張
  • Year and Date: 2008-11-15
• [Presentation] WIP is a chaperone for WASP (2008)
  • Author(s): Yoji Sasahara, Shigeru Tsuchiya, et.al.
  • Organizer: 第50回米国血液学会
  • Place of Presentation: サンフランシスコ(米国)