Expression of muscle cell surface antigen 5.1H11 in infantile or juvenile spinal muscular atrophy.
Monoclonal antibody 5.1H11 reacts with a cell surface antigen of human myoblasts and myotubes in cell culture. We found that levels of this antigen decrease to background levels during development of fetal muscle, and this down-regulation occurs after innervation. Adult myofibers are uniformly negative for 5.1H11 reactivity. Denervated muscle fibers from cases of Werdnig-Hoffmann disease and Kugelberg-Welander syndrome expressed 5.1H11 reactivity, while innervated fibers on the same sections were negative. Levels of 5.1H11 antigen therefore appear to be regulated by nerve in these spinal muscular atrophies.
D000954:Antigens, Surface / Q000032:analysis*; D006801:Humans; D009133:Muscular Atrophy / Q000378:metabolism*
D000954:Antigens, Surface