ArticleThe Journal of clinical investigation2015
Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies.
Article in The Journal of clinical investigation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 49 citations in OpenAlex.
- Reducing Body Myopathy in Female Patients WithNeurology. Genetics · 2026Article
- Recent advances in immunological mechanisms and murine disease models of idiopathic inflammatory myopathies.Inflammation and regeneration · 2025Review
- Integrative Approaches to Myopathies and Muscular Dystrophies: Molecular Mechanisms, Diagnostics, and Future Therapies.International journal of molecular sciences · 2025Review
- Circulating immunoregulatory B cell and autoreactive antibody profiles predict lack of toxicity to anti-PD-1 checkpoint inhibitor treatment in advanced melanoma.Journal for immunotherapy of cancer · 2025Article
- Anti-FHL1 autoantibodies in adult patients with myositis: a longitudinal follow-up analysis.Rheumatology (Oxford, England) · 2025Article
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- Article
- Role of four and a half LIM domain protein 1 in tumors (Review).Oncology letters · 2025Review
- Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.Rheumatology (Oxford, England) · 2023Article
- Autoantibodies against four-and-a-half-LIM domain 1 (FHL1) in inflammatory myopathies: results from an Australian single-centre cohort.Rheumatology (Oxford, England) · 2022Article
- The Impact of Dysphagia in Myositis: A Systematic Review and Meta-Analysis.Journal of clinical medicine · 2020Review
- Four and a half LIM domains protein 1 can be as a double-edged sword in cancer progression.Cancer biology & medicine · 2020Review
- Interleukin-37 (IL-37) Suppresses Pertussis Toxin-Induced Inflammatory Myopathy in a Rat Model.Medical science monitor : international medical journal of experimental and clinical research · 2018Article
- T-cell transcriptomics from peripheral blood highlights differences between polymyositis and dermatomyositis patients.Arthritis research & therapy · 2018Article
- The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy.Clinical rheumatology · 2018Article
- Bench to bedside review of myositis autoantibodies.Clinical and molecular allergy : CMA · 2018Review
- Myositis in 2016: New tools for diagnosis and therapy.Nature reviews. Rheumatology · 2017Review
- Diagnosis and classification of idiopathic inflammatory myopathies.Journal of internal medicine · 2016Review
- Inflammatory myopathies: Anti-FHL1 antibodies linked to IIM.Nature reviews. Rheumatology · 2016Article
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Authors and funding
26 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations of the gene encoding four-and-a-half LIM domain 1 (FHL1) are the causative factor of several X-linked hereditary myopathies that are collectively termed FHL1-related myopathies. These disorders are characterized by severe muscle dysfunction and damage. Here, we have shown that patients with idiopathic inflammatory myopathies (IIMs) develop autoimmunity to FHL1, which is a muscle-specific protein. Anti-FHL1 autoantibodies were detected in 25% of IIM patients, while patients with other autoimmune diseases or muscular dystrophies were largely anti-FHL1 negative. Anti-FHL1 reactivity was predictive for muscle atrophy, dysphagia, pronounced muscle fiber damage, and vasculitis. FHL1 showed an altered expression pattern, with focal accumulation in the muscle fibers of autoantibody-positive patients compared with a homogeneous expression in anti-FHL1-negative patients and healthy controls. We determined that FHL1 is a target of the cytotoxic protease granzyme B, indicating that the generation of FHL1 fragments may initiate FHL1 autoimmunity. Moreover, immunization of myositis-prone mice with FHL1 aggravated muscle weakness and increased mortality, suggesting a direct link between anti-FHL1 responses and muscle damage. Together, our findings provide evidence that FHL1 may be involved in the pathogenesis not only of genetic FHL1-related myopathies but also of autoimmune IIM. Importantly, these results indicate that anti-FHL1 autoantibodies in peripheral blood have promising potential as a biomarker to identify a subset of severe IIM.
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