Alteration of snRNAs in amyotrophic lateral sclerosis

• Project/Area Number: 23890060
• Research Category: Grant-in-Aid for Research Activity Start-up
• Allocation Type: Single-year Grants
• Research Field: Neurology
• Research Institution: Niigata University
• Principal Investigator: ISHIHARA Tomohiko 新潟大学, 脳研究所神経内科, 助教 (70612232)
• Project Period (FY): 2011 – 2012
• Project Status: Completed (Fiscal Year 2012)
• Budget Amount: ¥3,250,000 (Direct Cost: ¥2,500,000、Indirect Cost: ¥750,000); Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2011: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
• Keywords: ALS / snRNA / U12 snRNA / スプライシング / 逆転写定量PCR / ｓｎRNA / U12 / 内在性コントロール

Research Abstract

We stated and investigated the hypothesis that the dysfunction of TDP-43 causesreduction of snRNAs in amyotrophic lateral sclerosis (ALS). We revealed thedownregulation of U12 snRNA in the tissues affected with ALS and not in non-affectedtissues. Several U12-type intron splicing tended to be downregulated in ALS affectedtissues and culture cells with depleted TDP-43.

Research Products

• [Journal Article] Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS (2012)
  • Author(s): Shiga,A. Ishihara,T.Onodera,O. et al
  • Journal Title: PLoS One Volume: vol.2
  • Peer Reviewed
• [Journal Article] Alteration of P0LDIP3 Splicing Associated with Loss of Function of TDP-43 in Tissues Affected with ALS (2012)
  • Author(s): Shiga A, et al ;
  • Journal Title: PLoS ONE Volume: 7(8) Issue: 8 Pages: e43120-e43120
  • DOI: 10.1371/journal.pone.0043120
  • Peer Reviewed
• [Presentation] Reduction of U12 snRNAs in nervous tissues with TDP-43 pathology in amyotrophic lateral sclerosis (2012)
  • Author(s): 石原智彦
  • Organizer: 第42回米国神経科学会
  • Year and Date: 2012-10-15
• [Presentation] ALS患者神経組織ではスプライシング関連機能性RNAが低下する (2012)
  • Author(s): 石原智彦
  • Organizer: 第53回神経学会総会
  • Year and Date: 2012-05-23
• [Presentation] ALS患者神経組織では スプライシング関連 機能性RNAが低下する (2012)
  • Author(s): 石原 智彦
  • Organizer: 第53回 神経学会総会
  • Place of Presentation: 東京国際フォーラム
• [Presentation] ALSにおけるGEM小体の異常： SMNとの関連 (2012)
  • Author(s): 石原 智彦
  • Organizer: 平成24年度 神経変性疾患に関する調査研究班 班会議
  • Place of Presentation: 東京 都市センターホテル
• [Presentation] Reduction of U12 snRNAs in nervous tissues with TDP-43 pathology in amyotrophic lateral sclerosis (2012)
  • Author(s): 石原 智彦
  • Organizer: 第42回 米国神経科学会
  • Place of Presentation: 米国 ニューオーリンズ
• [Presentation] AlterationofsnRNAsinamyotrophiclateralsclerosis (2011)
  • Author(s): 石原智彦
  • Organizer: 第41回米国神経科学会
  • Year and Date: 2011-11-12
• [Presentation] Alteration of snRNAs in amyotrophic lateral sclerosis. (2011)
  • Author(s): 石原 智彦
  • Organizer: 第41回 米国神経科学会
  • Place of Presentation: ワシントン（USA）