Approach to cerebral small vessel disease: lesson from CARASIL.

2015 Fiscal Year Final Research Report

• Project/Area Number: 25293200
• Research Category: Grant-in-Aid for Scientific Research (B)
• Allocation Type: Partial Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Niigata University
• Principal Investigator: Onodera Osamu 新潟大学, 脳研究所, 教授 (20303167)
• Co-Investigator(Kenkyū-buntansha): SATO Toshiya 北里大学, 医学部, 教授 (90359703) ; TOYOSHIMA Yasuko 新潟大学, 脳研究所, 准教授 (20334675) ; KOYAMA Akihide 新潟大学, 研究推進機構, 助教 (90622209)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: 脳血管

Outline of Final Research Achievements

Cerebral small-vessel disease (CSVD) is a neurological disorder involving white matter lesions. CSVD is frequently observed in an elderly population and causes cognitive impairment and motor dysfunction. However little is known about a molecular pathogenesis for CSVD. Cerebral autosomal-recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL), an autosomal-recessive inherited cerebral small vessel disease (CSVD), involves severe leukoaraiosis, multiple lacunar infarcts, early-onset alopecia, and spondylosis deformans without hypertension. High-temperature requirement serine peptidase A1 (HTRA1) gene mutations cause CARASIL by decreasing HTRA1 protease activity. We have investigated the HTRA1 deficit mouse. We found that these mice showed mural cell and internal elastic membrane degeneration. We found fibronectin and protein X accumulates at the internal membrane. The pathological findings of the mouse resemble those of CARASIL as well as sporadic CSVD.

Free Research Field

神経内科学