AI Article Synopsis
- The study explores how antibodies to signal recognition particle (SRP) may lead to necrotizing myopathy, focusing on the role of anti-SRP+serum and complement on myoblast cell viability.
- Cell viability was minimally affected by anti-SRP+serum alone, but significantly decreased when fresh complement was added.
- Immunostaining showed the presence of SRP and complement components in both cultured cells and muscle biopsies from affected patients, suggesting a complement-dependent mechanism in this type of myopathy.
Article Abstract
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.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jneuroim.2013.08.008 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Anti-SRP myositis: a diagnostic and therapeutic challenge.
Turk J Pediatr
December 2024
Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Türkiye.
Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.
Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels.
Anti-SRP Antibodies and Myocarditis in Systemic Sclerosis Overlap Syndrome with Immune-Mediated Necrotizing Myositis (IMNM).
Medicina (Kaunas)
October 2024
Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
Article Synopsis
- - Overlap syndrome of systemic sclerosis and idiopathic inflammatory myopathies is becoming more common, but its link with immune-mediated necrotizing myositis is not well-documented, requiring careful differentiation for proper treatment and prognosis.
- - Anti-signal recognition particle (anti-SRP) antibodies are crucial in diagnosing immune-mediated necrotizing myopathy, causing muscle damage and weakness, while their relationship with cardiac issues remains inconclusive, complicating potential management.
- - Diagnosing heart complications like myocarditis in these patients is challenging, often relying on cardiac imaging rather than biopsies, and this paper explores the issues of accurately diagnosing and treating diseases related to collagen and anti-SRP antibodies.
Comprehensive Enteroviral Serology Links Infection and Anti-Melanoma Differentiation-Associated Protein 5 Dermatomyositis.
ACR Open Rheumatol
January 2025
Johns Hopkins University School of Medicine, Baltimore, Maryland.
Objective: Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous, systemic autoimmune diseases characterized by specific clinical features and, frequently, skeletal muscle inflammation. Specific subtypes of IIMs can be characterized by myositis-specific autoantibodies and are associated with distinct clinical phenotypes. Here, we focus on anti-melanoma differentiation-associated protein 5 (MDA5)-positive myositis and anti-signal recognition particle (SRP)-positive myositis, both of which exhibit seasonality but lack known environmental triggers.
View Article and Find Full Text PDFClinical characteristics of idiopathic inflammatory myopathies related to anti-SRP: a single center experience.
Sci Rep
October 2024
Department of Rheumatology and Immunology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, People's Republic of China.
Idiopathic inflammatory myopathy (IIM) with autoantibodies recognizing the signal recognition particle (SRP) is characterized by prominent proximal weakness, infrequent extramuscular involvement, dramatically elevated creatine kinase levels, and myofiber necrosis with few inflammatory cell infiltrates in muscle tissue. To enhance understanding of the clinically used diagnosis and treatment of this disease, this study presents a single-center experience and analysis of a Chinese cohort with anti-SRP IIM. The most recent European Neuromuscular Center criteria were used to include Anti-SRP IIM patients from September 2016 to November 2019.
View Article and Find Full Text PDFINPATIENT REHABILITATION AND FUNCTIONAL OUTCOME OF A CASE OF ANTI-SRP IMMUNE-MEDIATED NECROTIZING MYOPATHY.
J Rehabil Med Clin Commun
October 2024
From the Department of Rehabilitation Medicine, Changi General Hospital, Singapore.
Objective: Immune-mediated necrotizing myopathy associated with anti-signal recognition particle antibodies is a rare and debilitating condition characterized by significant muscle weakness and resultant disability. Although there are existing recommendations for physical therapy and exercise for patients with myositis in current literature, effective rehabilitation guidelines for such patients have not been fully established.
Case Report: A 42-year-old woman presented with subacute onset proximal upper and lower limb weakness and dysphagia.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!