in vivo imaging of transcytosis of lysosomal enzymes

• Project/Area Number: 24590249
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: General anatomy (including Histology/Embryology)
• Research Institution: Kagoshima University (2014); Niigata University (2012-2013)
• Principal Investigator: SHIBATA Masahiro 鹿児島大学, 医歯(薬)学総合研究科, 教授 (10343253)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Project Status: Completed (Fiscal Year 2014)
• Budget Amount: ¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2013: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000); Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
• Keywords: リソソーム / カテプシン / カテプシンD / 鶏胚 / ライソゾーム病

Outline of Final Research Achievements

Lysosomal Storage Diseases (LSDs) are a group of congenital metabolic disorders caused by lysosomal dysfunction. Enzyme replacement therapy which has been adopted to several LSDs has no effect on neurons due to the existence of blood-brain barrier. To elucidate the mechanism of the transcytosis of lysosomal enzymes in nervous system, we developed a chick model to monitor lysosomal enzymes in vivo using a two-photon laser scanning microscopy.

Research Products

• [Journal Article] Comparative study of the shell development of hard- and soft-shelled turtles (2014)
  • Author(s): Nagashima H, Shibata M, Taniguchi M, Ueno S, Kamezaki N, Sato N
  • Journal Title: J Anat Volume: in press
  • Peer Reviewed
• [Journal Article] Biogenesis and Proteolytic Processing of Lysosomal DNase II. (2013)
  • Author(s): Ohkouchi, S., Shibata, M., Sasaki, M., Koike, M., Safig, P., Peters, C., Nagata, S., and Uchiyama, Y.
  • Journal Title: PloS One Volume: 8 Issue: 3 Pages: e59148-e59148
  • DOI: 10.1371/journal.pone.0059148
  • Peer Reviewed
• [Journal Article] Differences in expression patterns of cathepsin C/dipeptidyl peptidase I in normal, pathological and aged mouse central nervous system. (2013)
  • Author(s): Koike M, Shibata M, Ezaki J, Peters C, Saftig P, Kominami E, Uchiyama Y.
  • Journal Title: Eur J Neurosci. Volume: 37(5) Issue: 5 Pages: 816-830
  • DOI: 10.1111/ejn.12096
  • Peer Reviewed
• [Journal Article] Resveratrol affects undifferentiated and differentiated PC12 cells differently, particularly with respect to possible differences in mitochondrial and autophagic fuctions (2013)
  • Author(s): Hyakawa N., Shiozaki M., Shibata M., Koike M., Uchiyama Y., Matsuura N., Gotow T.
  • Journal Title: Eur. J. Cell Biol Volume: 92 Issue: 1 Pages: 30-43
  • DOI: 10.1016/j.ejcb.2012.10.002
  • Peer Reviewed
• [Presentation] ISLR2, a novel marker for longitudinal tracts in early embryonic forebrain (2014)
  • Author(s): Shunsaku Homma, Takako Shimada, Masahiro Shibata, Noboru Sato, Hiroyuki Yaginuma
  • Organizer: 日本神経科学会
  • Place of Presentation: 横浜市（パシフィコ横浜）
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] Selective autophagy of lysosomes with ceroid-lipofuscin in neurons deficient in cathepsinD (2014)
  • Author(s): Tomohisa Nanao, Koike Masato, Yamaguchi Junji, Shibata Masahiro, Uchiyama Yasuo
  • Organizer: 日本神経科学会
  • Place of Presentation: 横浜市（パシフィコ横浜）
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] Hippocampal pyramidal neurons lacking LC3A and LC3B are resistant to hypoxic-ischemic brain injury in neonatal mice (2014)
  • Author(s): Junji Yamaguchi, Sunabori Takehiko, Koike Masato, Nanao Tomohisa, Shibata Masahiro, Uchiyama Yasuo
  • Organizer: 日本神経科学会
  • Place of Presentation: 横浜市（パシフィコ横浜）
  • Year and Date: 2014-09-11 – 2014-09-13
• [Presentation] 変異体カテプシンＤによる鶏胚の神経細胞死 (2014)
  • Author(s): 柴田昌宏、相馬健一、佐藤昇
  • Organizer: 日本解剖学会
  • Place of Presentation: 栃木県下野市
• [Presentation] Development of a novel model for polyglutamine pathogenesis using the chicken embryo (2013)
  • Author(s): Shibata M, Nakayama H, Yasudo M, Ito K, Uemura O, Okamoto H, Sato N
  • Organizer: 日本神経科学会
  • Place of Presentation: 京都市
• [Presentation] 鶏胚を用いたリソソームカテプシンによる細胞死誘導モデルの構築 (2013)
  • Author(s): 柴田昌宏、伊藤健二朗、佐藤昇
  • Organizer: 第１１８回日本解剖学会全国学術集会
  • Place of Presentation: 香川県高松市