The development and analysis of selective neuronal pathology

2014 Fiscal Year Final Research Report

• Project Area: Generation of synapse-neurocircuit pathology
• Project/Area Number: 22110004
• Research Category: Grant-in-Aid for Scientific Research on Innovative Areas (Research in a proposed research area)
• Allocation Type: Single-year Grants
• Review Section: Biological Sciences
• Research Institution: Juntendo University (2012-2014); The Institute of Physical and Chemical Research (2010-2011)
• Principal Investigator: NUKINA Nobuyuki 順天堂大学, 医学(系)研究科(研究院), 客員教授 (10134595)
• Co-Investigator(Kenkyū-buntansha): MIYAZAKI Haruko 独立行政法人理化学研究所, 視床発生研究チーム, 研究員 (80525890) ; YAMANAKA Tomoyuki 独立行政法人理化学研究所, 視床発生研究チーム, 研究員 (00381575) ; MATSUMOTO Gen 独立行政法人理化学研究所, 視床発生研究チーム, 研究員 (50415303)
• Project Period (FY): 2010-04-01 – 2015-03-31
• Keywords: ポリグルタミン病 / 大脳基底核 / FUS/TLS / p62 / NF-Y / セルソーター

Outline of Final Research Achievements

To elucidate the mechaism of selective neuronal degeneration of Huntington disease, we analyzed aggregate interacting proteins including FUS/TLS,NF-YA,and p62 using knockout mice and showed their in vivo functions.Investigating sodium channel beta4 subunit using knockout mouse, we demonstrated the resurgent current is regulated by beta4 in medium spiny neurons(MSNs) and during this study, we found the MSNs have unmyelinated fibers, which was first clearly demonstrated in this study.We established the methods to analyze the gene expressions of selected neurons by FACS and found new genes which changed in HD MSNs.

Free Research Field

病態脳科学、神経内科学