The multifaceted character of lymphotoxin ¦Â in inflammatory myopathies and muscular dystrophies

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• 作者：Kim K. Creus ; Boel De Paepe ; Joachim Weis ; Jan L. De Bleecker
• 关键词：Lymphotoxin ¦Â ; Inflammatory myopathy ; Dermatomyositis ; Polymyositis ; Sporadic inclusion body myositis ; Muscular dystrophy
• 刊名：Neuromuscular Disorders
• 出版年：2012
• 出版时间：August, 2012
• 年：2012
• 卷：22
• 期：8
• 页码：712-719
• 全文大小：1104 K

文摘

Lymphotoxin beta (LT¦Â) regulates some inflammatory mechanisms that could be operative in idiopathic inflammatory myopathies (IM). We studied LT¦Â and LT¦ÂR in inflammatory myopathies, normal and disease controls with immunohistochemistry, Western blotting and in situ hybridisation. LT¦Â occurs in myonuclei of normal controls, implying its role in normal muscle physiology. LT¦Â is strongly upregulated in regenerating muscle fibres in all myopathies, but not in denervated myofibres. Normal-appearing myofibres in inflammatory myopathies and muscular dystrophies express LT¦Â possibly reflecting early myofibre damage, representing a hitherto undescribed pathologic hallmark. Furthermore, we visualised LT¦Â in several inflammatory cell types in inflammatory myopathies, suggesting its involvement in the different inflammatory mechanisms underlying inflammatory myopathy subgroups.