ArticleInternational journal of clinical and experimental pathology2015
CD4+ cells, macrophages, MHC-I and C5b-9 involve the pathogenesis of dysferlinopathy.
Article in International journal of clinical and experimental pathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 36 citations in OpenAlex.
- Insights into the heterogeneous muscle lipidome of dysferlin-deficient mice: effects of age, muscle type, and sex.Skeletal muscle · 2026Article
- Emerging atypical clinicopathological manifestations of immune-mediated necrotizing myopathy (IMNM).Neuromuscular disorders : NMD · 2025Review
- Comprehensive Proteomic Analysis of Dysferlinopathy Unveiling Molecular Mechanisms and Biomarkers Linked to Pathological Progression.CNS neuroscience & therapeutics · 2024Article
- The Dysferlinopathies Conundrum: Clinical Spectra, Disease Mechanism and Genetic Approaches for Treatments.Biomolecules · 2024Review
- Becker Muscular Dystrophy Accompanied by Anti-HMGCR Antibody-positive Immune-mediated Necrotizing Myopathy.Internal medicine (Tokyo, Japan) · 2023Article
- Pediatric immune-mediated necrotizing myopathy.Frontiers in neurology · 2023Review
- Inhibition of the immunoproteasome modulates innate immunity to ameliorate muscle pathology of dysferlin-deficient BlAJ mice.Cell death & disease · 2022Article
- Clinical and pathological features of immune-mediated necrotising myopathies in a single-centre muscle biopsy cohort.BMC musculoskeletal disorders · 2022Article
- Novel compound heterozygous mutations in the TTN gene: elongation and truncation variants causing limb-girdle muscular dystrophy type 2J in a Han Chinese family.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
- Oxidative Stress, Inflammation and Connexin Hemichannels in Muscular Dystrophies.Biomedicines · 2022Review
- The inflammatory pathology of dysferlinopathy is distinct from calpainopathy, Becker muscular dystrophy, and inflammatory myopathies.Acta neuropathologica communications · 2022Article
- Key biomarkers and latent pathways of dysferlinopathy: Bioinformatics analysis andFrontiers in neurology · 2022Article
- Article
- The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases.Degenerative neurological and neuromuscular disease · 2021Article
- Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies.Frontiers in immunology · 2021Review
- Immune-mediated necrotizing myopathy: clinical features and pathogenesis.Nature reviews. Rheumatology · 2020Review
- Functions of Vertebrate Ferlins.Cells · 2020Review
- Disease-specific and glucocorticoid-responsive serum biomarkers for Duchenne Muscular Dystrophy.Scientific reports · 2019Article
- Immunobiology of Inherited Muscular Dystrophies.Comprehensive Physiology · 2018Review
- Molecular Therapies for Muscular Dystrophies.Current treatment options in neurology · 2018Review
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveDysferlin is a sarcolemmal protein that plays an important role in membrane repair by regulating vesicle fusion with the sarcolemma. Mutations in the dysferlin gene (DYSF) lead to multiple clinical phenotypes, including Miyoshi myopathy (MM), limb girdle muscular dystrophy type 2B (LGMD 2B), and distal myopathy with anterior tibial onset (DMAT). Patients with dysferlinopathy also show muscle inflammation, which often leads to a misdiagnosis as inflammatory myopathy. In this study, we examined and analyzed the dyferlinopathy-associated immunological features.
methodsComparative immunohistochemical analysis of inflammatory cell infiltration, and muscle expression of MHC-I and C5b-9 was performed using muscle biopsy samples from 14 patients with dysferlinopathy, 7 patients with polymyositis, and 8 patients with either Duchenne muscular dystrophy or Becker muscular dystrophy (DMD/BMD).
resultsImmunohistochemical analysis revealed positive staining for immune response-related CD4+ cells, macrophages, MHC-I and C5b-9 in dysferlinopathy, which is in a different mode of polymyositis and DMD/BMD.
conclusionThese results demonstrated the involvement of immune factors in the pathogenesis of dysferlinopathy.
Indexed as
Identifiers
26045819PMC4440128W2431744265What OpenQuestion holds
Registered trials
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