De Paepe Boel, Schröder J Michael, Martin Jean-Jacques, Racz Gabor Z, De Bleecker Jan L
Department of Neurology, Ghent University Hospital, De Pintelaan 185, B-9000 Ghent, Belgium.
Neuromuscul Disord. 2004 Apr;14(4):265-73. doi: 10.1016/j.nmd.2004.01.001.
We studied the distribution of stromal cell-derived factor 1 isoforms alpha and beta, and their receptor CXCR4, in polymyositis, sporadic inclusion body myositis and dermatomyositis using in situ hybridization, immunohistochemistry, immunofluorescence and Western blotting. In control muscle, polymyositis and sporadic inclusion body myositis, stromal cell-derived factor-1alpha expression was noted in muscle fibers, while stromal cell-derived factor-1beta and CXCR4 were predominantly localized to capillaries and arterioles. In dermatomyositis, stromal cell-derived factor-1beta immunoreactivity of blood vessels was focally increased. The vast majority of inflammatory cells in idiopathic inflammatory myopathies were CXCR4 positive. A subset of helper T-cells and macrophages expressed stromal cell-derived factor-1alpha, while only rare inflammatory cells expressed stromal cell-derived factor-1beta. A significant increase of stromal cell-derived factor-1alpha and CXCR4 was observed in protein extracts of idiopathic inflammatory myopathies in comparison with normal controls. The abundance of both CXCR4 and its ligand stromal cell-derived factor-1 implicates their interaction in the pathogenesis of idiopathic inflammatory myopathies and identifies these proteins as possible targets for selective immune therapy.
我们运用原位杂交、免疫组织化学、免疫荧光和蛋白质印迹法，研究了基质细胞衍生因子1α和β亚型及其受体CXCR4在多发性肌炎、散发性包涵体肌炎和皮肌炎中的分布情况。在对照肌肉、多发性肌炎和散发性包涵体肌炎中，可见基质细胞衍生因子-1α在肌纤维中表达，而基质细胞衍生因子-1β和CXCR4主要定位于毛细血管和小动脉。在皮肌炎中，血管的基质细胞衍生因子-1β免疫反应性呈局灶性增加。特发性炎性肌病中的绝大多数炎性细胞CXCR4呈阳性。一小部分辅助性T细胞和巨噬细胞表达基质细胞衍生因子-1α，而仅有罕见的炎性细胞表达基质细胞衍生因子-1β。与正常对照相比，在特发性炎性肌病的蛋白质提取物中观察到基质细胞衍生因子-1α和CXCR4显著增加。CXCR4及其配体基质细胞衍生因子-1的丰度表明它们在特发性炎性肌病的发病机制中相互作用，并确定这些蛋白质为选择性免疫治疗的可能靶点。
