Analysis of Neurodegenerative gene product, DRPLA protein

• Project/Area Number: 19500266
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neuroscience in general
• Research Institution: The University of Tokyo
• Principal Investigator: DATE Hidetoshi The University of Tokyo, 医学部附属病院, 特任助教 (30401037)
• Project Period (FY): 2007 – 2009
• Project Status: Completed (Fiscal Year 2009)
• Budget Amount: ¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000); Fiscal Year 2009: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000); Fiscal Year 2008: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000); Fiscal Year 2007: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
• Keywords: 分子・神経細胞科学 / ポリグルタミン病 / 天然変性タンパク質 / エピジェネティクス / 分子・細胞神経科学

Research Abstract

Dentatorubral-pallidoluysian atrophy (DRPLA ; MIM125370) is an autosomal dominant neurodegenerative disorder caused by unstable expansion of a CAG trinucleotide repeat in exon 5 which codes for a polyglutamine stretch. Nowadays, there is no treatment ways for DRPLA patient, because DRPLA protein fuction remains unclear. To develop for the therapy for DRPLA, we examined DRPLA protein biochemical function deeply.

Research Products

• [Journal Article] Intranuclear degradation of polyglutamine aggregates by the ubiquitin-proteasome system (2009)
  • Author(s): Iwata A, Nagashima Y, Matsumoto L, Suzuki T, Yamanaka T, Date H, Deoka K, Nukina N, Tsuji S.
  • Journal Title: J Biol Chem. 284(15) Pages: 9796-803
  • Peer Reviewed
• [Journal Article] SNP HiTLink : a high-throughput linkage analysis system employing dense SNP data (2009)
  • Author(s): Yoko Fukuda, Yasuo Nakahara, Hidetoshi Date, Yuji Takahashi, Jun Goto, Akinori Miyashita, Ryozo Kuwano, Hiroki Adachi, Eiji Nakamura, Shoji Tsuji
  • Journal Title: BMC Bioinformatics 10 Pages: 121-121
  • Peer Reviewed
• [Journal Article] Mutations for Gaucher disease confer high susceptibility to Parkinson disease (2009)
  • Author(s): Mitsui J, Mizuta I, Toyoda A, Ashida R, Takahashi Y, Goto J, Fukuda Y, Date H, Iwata A, Yamamoto M, Hattori N, Murata M, Toda T, Tsuji S.
  • Journal Title: Arch Neurol 66(5) Pages: 571-6
  • Peer Reviewed
• [Journal Article] Intranuclear degradation of polyglutamine aggregates by the ubiquitin-proteasome system. (2009)
  • Author(s): Iwata A, 他8名
  • Journal Title: Journal of Biological Chemistory 284 Pages: 9796-9803
  • Peer Reviewed
• [Journal Article] Mutations for Gaucher disease confer high susceptibility to Parkinson disease. (2009)
  • Author(s): Mitsui J, 他13名
  • Journal Title: Archives of Neurology 66 Pages: 571-576
  • Peer Reviewed
• [Journal Article] SNP HiTLink : a high-throughput linkage analysis system employing dense SNP data. (2009)
  • Author(s): Fukuda Y, 他9名
  • Journal Title: BMC Bioinfomatics 24 Pages: 121-121
  • Peer Reviewed
• [Journal Article] 1ntra-nuclear degradation of polyglutamine aggregates by the ubiquitin proteasome system. (2009)
  • Author(s): Atsushi Iwata
  • Journal Title: Journal of Biological Chemistry in press
  • Peer Reviewed
• [Journal Article] Aprataxin, causative gene product for EAOH/AOA1, repairs DNA single-strand breaks with damaged 3'-phosphate and 3'-phosphoglycolate ends (2007)
  • Author(s): Takahashi T, Tada M, Igarashi S, Koyama A, Date H, Yokoseki A, Shiga A, Yoshida Y, Tsuji S, Nishizawa M, Onodera O.
  • Journal Title: Nucleic Acids Res. 35(11) Pages: 3797-809
  • Peer Reviewed
• [Journal Article] IFN A-1b may severely exacerbate Japanese optico-spinal MS in neuromyelitis optica spectrum
  • Author(s): Jun Shimizu, Yuki Hatanaka, Michiko Hasegawa, Atsushi Iwata, Izumi Sugimoto, Hidetoshi Date, Jun Goto, Teruo Shimizu, Masami Takatsu, Yasuhisa Sakurai, Hirofumi Nakase, Yoshikazu Uesaka, Hideji Hashida, Tadashi Komiya, Shoji Tsuji
  • Journal Title: NEUROLOGF (in press)
  • Peer Reviewed
• [Presentation] DRPLA 治療薬探索を目指した Cell-based high throughput screening (2010)
  • Author(s): 伊達英俊
  • Organizer: 日本神経学総会
  • Place of Presentation: 東京国際フォーラム (東京)
  • Year and Date: 2010-05-20