Pathologcial analysis for mouse model showing dystonia

2015 Fiscal Year Final Research Report

• Project/Area Number: 24700351
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Nerve anatomy/Neuropathology
• Research Institution: Niigata University
• Principal Investigator: HORIE Masao 新潟大学, 医歯学系, 講師 (70322716)
• Project Period (FY): 2012-04-01 – 2016-03-31
• Keywords: ジストニア / ジストニン / マウス / 中枢神経系 / 末梢神経系

Outline of Final Research Achievements

Dystonia is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements, abnormal postures, or both. We had generated a novel Dystonin gene trap (DstGt) mice, in which actin-binding domain-containing isoforms are disrupted. Homozygous DstGt mice showed typical progressive neurological symptoms: severe motor disorders in their limbs and twisted postures. Electromyogram showed abnormal co-contractions of agonist and antagonist muscle in DstGt homozygotes. In histological analyses, abnormal neurofilament accumulation was observed in both peripheral and central nervous system. In order to know the abnormal neural regions possibly affecting the motor disorder in these mice, we mapped the distribution of abnormal neurofilament protein and found that it mainly concentrated in the motor-related neurons, suggesting that they are involved in the abnormal motor phenotype in DstGt.

Free Research Field

神経解剖学