期刊
JOURNAL OF NEUROIMMUNOLOGY
卷 264, 期 1-2, 页码 65-70出版社
ELSEVIER
DOI: 10.1016/j.jneuroim.2013.08.008
关键词
Anti-SRP antibody; Necrotizing myopathy; Complement mediated cell lysis
资金
- Enid and Arthur Home Memorial Scholarship and a University of Western Australia Scholarship for International Research Fees
- Neuromuscular Foundation of Western Australia
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. (C) 2013 Elsevier B.V. All rights reserved.
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