Complement-mediated muscle cell lysis: A possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM)

Complement-mediated muscle cell lysis: A possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM)

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. +. seru...

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Bibliographic Details
Main Authors: Arada Rojana-udomsart, Chalermchai Mitrpant, Christine Bundell, Loren Price, Yue Bei Luo, Victoria Fabian, Steve D. Wilton, Peter Hollingsworth, Frank L. Mastaglia
Other Authors: University of Western Australia
Format: Article
Published: 2018
Subjects:
Immunology and Microbiology
Medicine
Neuroscience
Online Access:https://repository.li.mahidol.ac.th/handle/123456789/31869
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Institution: Mahidol University
Description
Summary: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. © 2013 Elsevier B.V.

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