Louboutin J P, Villanova M, Ulrich G, De Clerck L S, Fardeau M, Sagniez M
URA CNRS 1340-Hôpital GR Laënnec, Nantes, France.
Muscle Nerve. 1996 Sep;19(9):1144-7. doi: 10.1002/(SICI)1097-4598(199609)19:9<1144::AID-MUS10>3.0.CO;2-V.
We have recently reported a French family presenting with an X-linked vacuolated myopathy. Here we show that levels of complement components C5 and C9 are elevated in the serum of these patients. Moreover, antitrypsin activity is decreased in the serum of the patients. Taken with the deposition of membrane attack complex encountered in muscle tissue, these results emphasize the role of complement in the pathogenesis of this rare muscular disorder.
我们最近报道了一个患有X连锁空泡性肌病的法国家庭。在此我们表明，这些患者血清中的补体成分C5和C9水平升高。此外，患者血清中的抗胰蛋白酶活性降低。结合在肌肉组织中遇到的膜攻击复合物沉积，这些结果强调了补体在这种罕见肌肉疾病发病机制中的作用。
