Caron A, Chapon F, Berthelin C, Viader F, Lechevalier B
Laboratory of Neuropathology, CHU Côte de Nâcre, Caen, France.
Neuromuscul Disord. 1993 Sep-Nov;3(5-6):541-6. doi: 10.1016/0960-8966(93)90112-w.
We report here for the first time positive anti-dystrophin labelling of inclusions in three cases belonging to the same family affected by familial cytoplasmic body myopathy (CBM). Inclusions are also stained, as reported previously, by anti-actin antibodies. The anti-desmin reaction was negative in the centre of cytoplasmic bodies (CB) but showed an enhancement of staining in the peripheral part. Abnormal sarcoplasmic staining of fibres with CB was also observed with that antibody. Anti-vimentin antibody labelling was negative. At present, the significance of this labelling by anti-dystrophin antibodies is unknown, but will open new fields for further investigations in an attempt to understand CB pathogenesis.
我们首次在此报告，在一个受家族性细胞质体肌病（CBM）影响的同一家族的三例病例中，包涵体出现抗肌萎缩蛋白阳性标记。如先前报道，包涵体也被抗肌动蛋白抗体染色。在细胞质体（CB）中心，抗结蛋白反应呈阴性，但在周边部分染色增强。用该抗体也观察到含有CB的纤维出现异常肌浆染色。抗波形蛋白抗体标记呈阴性。目前，抗肌萎缩蛋白抗体这种标记的意义尚不清楚，但将为进一步研究开辟新领域，以试图了解CB的发病机制。
