Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Antibodies, Monoclonal
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Biopsy
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Blotting, Western
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Clusterin
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Complement Inactivator Proteins / analysis
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Complement Inactivator Proteins / immunology
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Complement Membrane Attack Complex / analysis*
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Fluorescent Antibody Technique
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Genetic Linkage
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Glycoproteins / analysis
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Glycoproteins / immunology
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Humans
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Membrane Glycoproteins / analysis*
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Membrane Glycoproteins / immunology
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Middle Aged
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Molecular Chaperones*
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Muscle Fibers, Skeletal / chemistry*
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Muscle Fibers, Skeletal / pathology
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Muscular Diseases / genetics
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Muscular Diseases / metabolism*
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Muscular Diseases / pathology
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Vacuoles / pathology
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Vitronectin
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X Chromosome*
Substances
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Antibodies, Monoclonal
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CLU protein, human
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Clusterin
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Complement Inactivator Proteins
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Complement Membrane Attack Complex
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Glycoproteins
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Membrane Glycoproteins
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Molecular Chaperones