Increased expression of HLA ABC class I antigens by muscle fibres in Duchenne muscular dystrophy, inflammatory myopathy, and other neuromuscular disorders.
The distribution of HLA ABC class I antigens in human skeletal muscle obtained by needle biopsy was investigated by means of a monoclonal antibody (W6/32) and an immunoperoxidase technique. Five samples from normal individuals and twenty-nine from patients with various neuromuscular disorders were examined. Normal muscle fibres and those from patients with congenital muscular dystrophy expressed little or no class I antigens, whereas muscle fibres of patients with myositis and various X-linked muscular dystrophies showed consistently strong expression. In other neuromuscular diseases expression was more variable. The presence of class I antigens on diseased muscle fibres may render them susceptible to cytotoxic T cells; these antigens may thus have an important role in the destruction of muscle fibres.
Appleyard
S T
ST
Dunn
M J
MJ
Dubowitz
V
V
Rose
M L
ML
D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal; D002648:Child; D002675:Child, Preschool; D006680:HLA Antigens / Q000032:analysis*; D015234:HLA-A Antigens; D015235:HLA-B Antigens; D015236:HLA-C Antigens; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D009132:Muscles / Q000276:immunology* / Q000473:pathology; D009136:Muscular Dystrophies / Q000276:immunology* / Q000473:pathology; D009220:Myositis / Q000276:immunology* / Q000473:pathology; D009468:Neuromuscular Diseases / Q000276:immunology* / Q000473:pathology
D000911:Antibodies, Monoclonal; D006680:HLA Antigens; D015234:HLA-A Antigens; D015235:HLA-B Antigens; D015236:HLA-C Antigens