Characterization of the function of dystroglycan N-terminal domain and its related disorders in nervous system

2015 Fiscal Year Final Research Report

• Project/Area Number: 25430075
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurochemistry/Neuropharmacology
• Research Institution: Teikyo University
• Principal Investigator: Matsumura Kiichiro 帝京大学, 医学部, 教授 (50260922)
• Co-Investigator(Kenkyū-buntansha): Masaki Toshihiro 帝京科学大学, 医療科学部, 教授 (00585028) ; Hagiwara Hiroki 帝京科学大学, 医療科学部, 教授 (80276732)
• Co-Investigator(Renkei-kenkyūsha): Saito Fumiaki 帝京大学, 医学部, 准教授 (40286993)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: ジストログリカン / 糖鎖修飾 / 蛋白質プロセッシング / トランスジェニックマウス

Outline of Final Research Achievements

The N-terminal domain of α-dystroglycan (α-DG-N) is cleaved and secreted from many types of cells. However, the functional role of secreted α-DG-N is still unidentified. To elucidate this point, we generated transgenic mice overexpressing α-DG-N ubiquitously. Interestingly, we demonstrated that the reactivity of IIH6, an antibody which detects specific sugar chain structure of α-dystroglycan, was markedly reduced in skeletal muscle. In contrast, laminin binding activity was well preserved. Based on these results, we propose that the evaluation not only of IIH6 reactivity but also of the laminin binding activity is necessary for the clinical diagnosis of α-dystroglycanopathies.

Free Research Field

神経内科学