ArticleArchives of neurology2012
Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis.
Article in Archives of neurology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 114 papers, 2 of them syntheses that pooled it.
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Who cites it
114 citing papers in PubMed, 2 syntheses or guidelines pooled it, 341 citations in OpenAlex.
- Genome-wide meta-analysis of myasthenia gravis uncovers new loci and provides insights into polygenic prediction.Nature communications · 2024Pooled it
- Ephedrine for myasthenia gravis, neonatal myasthenia and the congenital myasthenic syndromes.The Cochrane database of systematic reviews · 2014Pooled it
- MuSK IgG4 autoantibodies cause myasthenia gravis by inhibiting binding between MuSK and Lrp4.Proceedings of the National Academy of Sciences of the United States of America · 2013Trial
- Prevalence and characteristics of patients with seronegative myasthenia gravis.Journal of neurology · 2026Article
- Low Prevalence and Inconsistency of LRP4-IgG Detection in Suspected Myasthenia Gravis: A Multicenter CBA Comparison.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Complement deposition, IgG subtyping and endplate destruction in LRP4-ab-positive myasthenia gravis.Acta neuropathologica communications · 2026Observational
- Nipocalimab Dose Selection in Generalized Myasthenia Gravis.CPT: pharmacometrics & systems pharmacology · 2025Article
- Effective use of BCMA-targeting bispecific T cell-engaging antibody in treatment-refractory LRP4Molecular therapy : the journal of the American Society of Gene Therapy · 2025Article
- Efgartigimod improves non-AChR generalized Myasthenia Gravis: a real world experience.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- [Treatment and clinical course of autoimmune myasthenia in Burkina Faso].Medecine tropicale et sante internationale · 2025Article
- Efficacy of MLN9708 (ixazomib) in experimental autoimmune myasthenia gravis and in anti-AChR producing primary thymic cell cultures from myasthenia gravis patients.Frontiers in immunology · 2025Article
- Thymus research in relation to myasthenia gravis: a new perspective on cell subpopulations and future directions.Frontiers in immunology · 2025Review
- Evaluation of nonmotor symptoms in myasthenia gravis patients.Turkish journal of medical sciences · 2025Article
- Clinical features of double seronegative ocular myasthenia gravis.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2024Article
- Neuronal LRP4 directs the development, maturation and cytoskeletal organization of Drosophila peripheral synapses.Development (Cambridge, England) · 2024Article
- Efgartigimod in generalized myasthenia gravis: A real-life experience at a national reference center.European journal of neurology · 2024Article
- Optimal time for the addition of non-corticosteroid immunosuppressants in myasthenia gravis: a single-center retrospective study in China.Frontiers in neurology · 2024Article
- Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background.Pathophysiology : the official journal of the International Society for Pathophysiology · 2023Review
- Review
- Neuronal LRP4 directs the development, maturation, and cytoskeletal organization of peripheral synapses.bioRxiv : the preprint server for biology · 2023Article
54 more citing papers are in PubMed but not listed here.
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo determine whether patients with myasthenia gravis (MG) have serum antibodies to lipoprotein-related protein 4 (LRP4), a newly identified receptor for agrin that is essential for neuromuscular junction formation, and to establish whether such antibodies contribute to MG pathogenesis.
designSerum samples from patients with MG with known status of serum antibodies to the acetylcholine receptor (AChR) and muscle-specific kinase (MuSK) and serum samples from control subjects (healthy individuals and individuals with other diseases) were tested for antibodies to LRP4. Serum samples with such antibodies were tested to determine whether they had the ability to inhibit 2 different functions of LRP4 at the neuromuscular junction.
settingSerum samples were collected at the Hellenic Pasteur Institute and Wayne State University. Samples were tested for LRP4 autoantibodies at Georgia Health Sciences University. Other immunoreactivities of the samples were tested at the Hellenic Pasteur Institute, Athens, Greece, or processed through University Laboratories of the Detroit Medical Center, Michigan. Patients The study included 217 patients with MG, 76 patients with other neurologic or psychiatric diseases, and 45 healthy control subjects.
resultsAnti-LRP4 antibodies were detected in 11 of 120 patients with MG without detectable anti-AChR or anti-MuSK antibodies (double seronegative) and in 1 of 36 patients without anti-AChR antibodies but with anti-MuSK antibodies, but they were not detected in any of the 61 patients with anti-AChR antibodies. No healthy control subjects and only 2 of the 76 control patients with neurologic disease had anti-LRP4 antibodies. Serum samples from patients with MG with anti-LRP4 antibodies were able to inhibit the LRP4-agrin interaction and/or alter AChR clustering in muscle cells.
conclusionsAnti-LRP4 antibodies were detected in the serum of approximately 9.2% of patients with double-seronegative MG. This frequency is intermediate compared with 2 recent studies showing anti-LRP4 antibodies in 2% and 50% of patients with double-seronegative MG from different geographic locations. Together, these observations indicate that LRP4 is another autoantigen in patients with MG, and anti-LRP4 autoantibodies may be pathogenic through different immunopathogenic processes.
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