S-protein is expressed in necrotic fibers in Duchenne muscular dystrophy and polymyositis.
Complement regulatory proteins (CD55, CD59) and a fluid-phase complement regulator (S-protein) expression and distribution were studied in Duchenne muscular dystrophy (DMD) and polymyositis by Western blots and immunocytochemistry. In muscle samples from control subjects, no specific signal was detected for CD55 or S-protein, and CD59 was present on the sarcolemma of the muscle fibers. In DMD and polymyositis, Western blots demonstrated a 18-20 kDa band corresponding to CD59, as well as a signal corresponding to S-protein. Immunocytochemistry showed a colocalization between complement membrane attack complex (MAC), a molecule previously demonstrated in necrotic muscle fibers in DMD and polymyositis, and S-protein in necrotic fibers of DMD and polymyositis. Necrotic muscle fibers were more numerous in muscle biopsies of DMD patients with stronger signals for S-protein in Western blots. These results suggest that S-protein is not able to prevent the full assembly of MAC in necrotic fibers of patients with DMD and polymyositis, but might instead inactivate MAC deposits present inside necrotic fibers or participate in the clearance of MAC-attacked muscle fibers.
Louboutin
Jean-Pierre
JP
Department of Medicine, Division of Medical Genetics, University of Pennsylvania School of Medicine, Wistar Institute, 3601 Spruce Street, Philadelphia, Pennsylvania 19104-4268, USA. jplouboutin@hotmail.com
Navenot
Jean-Marc
JM
Rouger
Karl
K
Blanchard
Dominique
D
D000911:Antibodies, Monoclonal; D000918:Antibody Specificity; D006801:Humans; D008562:Membrane Glycoproteins / Q000032:analysis / Q000096:biosynthesis* / Q000276:immunology; D018485:Muscle Fibers, Skeletal / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism* / Q000473:pathology; D009336:Necrosis; D017285:Polymyositis / Q000378:metabolism* / Q000473:pathology; D019096:Vitronectin
D000911:Antibodies, Monoclonal; D008562:Membrane Glycoproteins; D019096:Vitronectin