Human β-defensin-3 correlates with muscle fibre degeneration in idiopathic inflammatory myopathies:

Innate Immunity

Anne-KatrinGüttsches¹ 

关键词: Host defence peptide;    innate immunity;    sporadic inclusion body myositis;    polymyositis;    Toll-like receptors;    muscle disease pathophysiology;   

DOI  :  10.1177/1753425913481820

学科分类：生物科学（综合）

来源: Sage Journals

【 摘 要 】

Sporadic inclusion body myositis (sIBM) and polymyositis (PM) are characterized by muscle inflammation, with sIBM showing additional degenerative alterations. In this study we investigated human beta defensins and associated TLRs to elucidate the role of the innate immune system in idiopathic inflammatory myopathies (IIM), and its association with inflammatory and degenerative alterations. Expression levels of human beta-defensin (HBD)-1, HBD-2, HBD-3 and TLR2, 3, 4, 7 and 9 were analysed by quantitative real-time PCR in skeletal muscle tissue. Localization of HBD-3, collagen 6, dystrophin, CD8-positive T-cells, CD-68-positive macrophages, β-amyloid, the autophagy marker LC3, and TLR3 were detected by immunofluorescence and co-localization was quantified. HBD-3 and all TLRs except for TLR9 were overexpressed in both IIM with significant overexpression of TLR3 in sIBM. HBD-3 showed characteristic intracellular accumulations near deposits of β-amyloid, LC3 and TLR3 in sIBM, and was detected in inflammatory ...

【 授权许可】

CC BY