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X‐linked vacuolated myopathy: Membrane attack complex deposition on the surface membrane of injured muscle fibers is not accompanied by S‐protein
Author(s) -
Louboutin JeanPierre,
Navenot JeanMarc,
Villanova Marcello,
Rouger Karl,
Merlini Luciano,
Fardeau Michel
Publication year - 1998
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199807)21:7<932::aid-mus11>3.0.co;2-s
Subject(s) - myopathy , complement membrane attack complex , immunocytochemistry , complement system , cd59 , pathology , immunohistochemistry , biology , myocyte , membrane protein , immune system , microbiology and biotechnology , medicine , membrane , immunology , biochemistry
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 S‐protein 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. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:932–935, 1998.