X-linked vacuolated myopathy: Membrane attack complex deposition on the surface membrane of injured muscle fibers is not accompanied by S-protein
✍ Scribed by Jean-Pierre Louboutin; Jean-Marc Navenot; Marcello Villanova; Karl Rouger; Luciano Merlini; Michel Fardeau
- Publisher
- John Wiley and Sons
- Year
- 1998
- Tongue
- English
- Weight
- 119 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0148-639X
No coin nor oath required. For personal study only.
✦ Synopsis
We have studied the expression of S-protein on the muscle from patients with X-linked vacuolated myopathy [characterized by the deposition of the complement C5b-9 membrane attack complex (MAC) over abnormal muscle fibers] and controls by immunocytochemistry and immunoblotting. No expression was detected on muscle from controls and patients with X-linked vacuolated myopathy. These findings suggest that Sprotein does not render the MAC inactive in X-linked vacuolated myopathy. This situation may be due to the fact that the pathways of MAC activation and the expression of S-protein in X-linked vacuolated myopathy are different from the ones observed in ischemic and/or necrotic, or immune diseases. These results emphasize the role of the membrane complement regulatory proteins (i.e., CD59) in X-linked vacuolated myopathy.