The elucidation of molecular mechanisms of capillary dysfunction with dermatomyositis.

2020 Fiscal Year Final Research Report

• Project/Area Number: 18K07527
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 52020:Neurology-related
• Research Institution: Yamaguchi University
• Principal Investigator: Sano Yasuteru 山口大学, 大学院医学系研究科, 助教 (20379978)
• Project Period (FY): 2018-04-01 – 2021-03-31
• Keywords: 皮膚筋炎 / タイトジャンクション / 微小血管内皮細胞 / ペリサイト

Outline of Final Research Achievements

Dermatomyositis (DM) is an immune-mediated disorder that most prominently involves muscle and skin. The loss of tight junction (TJ) of some remaining microvasculature is a hallmark of DM. Firstly, we demonstrated the possibility that sera from DM patients bring apoptosis of endothelium derived from endomysial capillaries. Then, we disclosed that GDNF and bFGF released from human skeletal muscle pericyte cell (HSMPCT) line increase the barrier function of human skeletal muscle microvascular endothelial cell line, TSM15. In addition, we also demonstrated that activated vitamin D strengthen the barrier function of TSM15. These results indicate that bFGF and GDNF, as well as activated vitamin D, might ameliorate the pathophysiology of DM via restoring the fragile barrier of endomysial endothelium.

Free Research Field

脳神経内科学

Academic Significance and Societal Importance of the Research Achievements

本研究成果は，皮膚筋炎の新たな治療法開発へと発展する可能性を秘めている．また，我々が樹立したヒト骨格筋内皮細胞株とペリサイト株は骨格筋微小血管の生理学の理解に役立つだけでなくデュシェンヌ型筋ジストロフィーなど骨格筋微小血管に障害がみられる筋疾患の病態理解に役立つものと考えられる．