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Elevated levels of complement components C5 and C9 and decreased antitrypsin activity in the serum of patients with X-linked vacuolated myopathy

✍ Scribed by J.-P. Louboutin; M. Villanova; G. Ulrich; L.S. De Clerck; M. Fardeau; M. Sagniez


Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
384 KB
Volume
19
Category
Article
ISSN
0148-639X

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✦ Synopsis


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. 0 1996