IL-6 as a target for delay the progression of muscular dystrophy

2017 Fiscal Year Final Research Report

• Project/Area Number: 16K18385
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Nerve anatomy/Neuropathology
• Research Institution: Tokyo Medical University
• Principal Investigator: WADA EIJI 東京医科大学, 医学部, 助教 (60756948)
• Project Period (FY): 2016-04-01 – 2018-03-31
• Keywords: デュシェンヌ型筋ジストロフィー / 筋疾患 / IL-6 / 線維化 / 筋再生 / 筋衛星細胞 / 炎症

Outline of Final Research Achievements

Interleukin-6 (IL-6) is related to inflammation and fibrosis but is also known as a myokine in skeletal muscle. Elevated IL-6 levels in blood and skeletal muscle are observed in patients and model animals of Duchenne muscular dystrophy (DMD); however, its contribution to the progression of muscular dystrophy is unclear. This study aimed to evaluate the effects of IL-6 receptor (IL-6R) blockage on the muscle pathology in DMD model mice. Treatment of IL-6R blockage successfully inhibited IL-6 pathway in skeletal muscle and significantly increased regenerating fibers. A significant reduction of phosphorylated STAT3 in skeletal muscle of treated mice supported the effects of IL-6R blockage on promoting muscle regeneration. Consistently, decreased serum CK levels, enlarged muscle diameter and reduced fibrosis in quadriceps muscle were seen. These results indicate that anti IL-6R antibody is a potential therapy for dystrophic muscle particularly for activating skeletal muscle regeneration.

Free Research Field

筋ジストロフィー