Complement-mediated muscle cell lysis: A possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM)
Rojana-udomsart, A., Mitrpant, C., Bundell, C., Price, L., Luo, Y-B, Fabian, V., Wilton, S.D., Hollingsworth, P. and Mastaglia, F.L. (2013) Complement-mediated muscle cell lysis: A possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM). Journal of Neuroimmunology, 264 (1-2). pp. 65-70.
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The mechanism of necrotizing myopathy associated with antibodies to signal recognition particle (SRP) remains unclear. We investigated the effect of anti-SRP + serum and complement on cell viability in myoblast cultures. Cell viability was only slightly reduced by incubation with anti-SRP + 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-SRP + 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.
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