Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6020598 | Journal of Neuroimmunology | 2013 | 6 Pages |
â¢Anti-SRP positive sera are myotoxic in vitro in the presence of complementâ¢Surface staining for SRP, C3c and C5b-9 in anti-SRP treated myoblastsâ¢Sarcolemmal staining for SRP, C3c and C5b-9 in anti-SRP myopathy biopsiesâ¢Complement-dependent antibody-mediated mechanism of injury in anti-SRP myopathy
The mechanism of necrotizing myopathy associated with antibodies to signal recognition particle (SRP) remains unclear. We investigated the effect of anti-SRPÂ +Â serum and complement on cell viability in myoblast cultures. Cell viability was only slightly reduced by incubation with anti-SRPÂ +Â serum compared with control serum. However, the addition of fresh complement resulted in a marked reduction in cell survival. Surface immunostaining for SRP, C3c and C5b-9 was demonstrated in cultures pre-incubated with anti-SRPÂ +Â serum and complement, and in muscle biopsies from patients with myopathy. These findings provide further support for a complement-dependent antibody-mediated mechanism in anti-SRP associated myopathy.