EEstablishment of treatment target for ALS / FTLD

2017 Fiscal Year Final Research Report

• Project/Area Number: 15K09323
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Keio University
• Principal Investigator: Ito Daisuke 慶應義塾大学, 医学部(信濃町), 講師 (80286450)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Keywords: 筋萎縮性側策硬化症 / 前頭側頭葉変性症 / optineurin / ubiquilin2

Outline of Final Research Achievements

1) Two different molecules involved in familial ALS, UBQLN 2 and OPTN are both involved in endosomal vesicles involved in proteolysis, have the function of connecting enodosome and autopahgy, and formed a novel protein quality control mechanism in endosome. 2) In DRP (dipeptidere peat protein) by non-AUG (RAN) translation from abnormal elongation GGGGCC repetitive sequence, expression of poly-PR, which is strongly basic, significantly reduces gemini of coiled bodies and Cajal bodies into Hela cells and suggested that it reflects the pathology of ALS.

Free Research Field

神経内科