Title:
【発明の名称】CAGリピート病の治療薬
Document Type and Number:
Japanese Patent JP3012923
Kind Code:
B2
Abstract:
To elucidate the molecular mechanisms of "gain of toxic function" of expanded polyglutamine stretches in CAG repeat expansion diseases, the inventors established an expression system of full-length and truncated cDNAs for dentatorubral-pallidoluysian atrophy (DRPLA) and found that truncated DRPLA proteins containing the expanded polyglutamine stretch, but not the full-length protein, form peri- and intra-nuclear aggregates consisting of filaments and concomitant apoptosis. The apoptotic cell death was partially suppressed by transglutaminase inhibitors, cystamine and monodansyl cadaverine, raising the possibility of involvement of transglutaminase reaction. The results may provide a potential basis for the development of therapeutic measures for CAG repeat expansion diseases.
Inventors:
Shoji Tsuji
Application Number:
JP2773998A
Publication Date:
February 28, 2000
Filing Date:
January 26, 1998
Export Citation:
Assignee:
Niigata University
International Classes:
A61K31/145; A61K31/195; A61K45/00; A61P21/00; A61K38/00; A61P25/00; A61P43/00; (IPC1-7): A61K31/195; A61P25/00; A61P25/14; A61K31/13
Attorney, Agent or Firm:
Akihide Sugimura (8 outside)