The thymus is thought to play an important role in the pathogenesis of myasthenia gravis (MG), an autoimmune disease characterized by skeletal muscle weakness. However, its role remains a mystery. The studies described represent our efforts to determine how intrathymic expression of the neuromuscular type of acetylcholine receptors (nAChRs) is involved in the immunopathogenesis of MG. We review our work characterizing the expression of the alpha subunit of nAChR (nAChRalpha) in the thymus and advance a new hypothesis that examines the intrathymic expression of this autoantigen in disease pathogenesis.
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http://dx.doi.org/10.1196/annals.1254.027 | DOI Listing |
Front Pediatr
January 2025
Department of Dermatology, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang Province, China.
Alopecia areata (AA) is a common non-scarring hair loss condition whose specific pathogenesis is not yet fully understood. In children, AA often co-occurs with atopic dermatitis (AD), complicating treatment. Here, we report the case of a child with myasthenia gravis who had severe AA and moderate AD.
View Article and Find Full Text PDFBackground: Myasthenia gravis (MG) and idiopathic inflammatory myopathies (IIM) are autoimmune disorders that can co-occur, complicating diagnosis and treatment. The molecular mechanisms underlying this comorbidity are not well understood.
Objective: This study aims to identify common differentially expressed genes (co-DEGs) between MG and IIM to elucidate shared pathogenic pathways and potential therapeutic targets.
Lancet Neurol
February 2025
Department of Neurology, International University of Health and Welfare, Narita, Japan.
Background: Evidence from preclinical studies suggests that IL-6 signalling has the potential to modulate immunopathogenic mechanisms upstream of autoantibody effector mechanisms in patients with generalised myasthenia gravis. We aimed to assess the safety and efficacy of satralizumab, a humanised monoclonal antibody targeting the IL-6 receptor, in patients with generalised myasthenia gravis.
Methods: LUMINESCE was a randomised, double-blind, placebo-controlled, multicentre, phase 3 study at 105 sites, including hospitals and clinics, globally.
Lancet Neurol
February 2025
Janssen Research & Development, a Johnson & Johnson Company, Titusville, NJ, USA.
Background: Given burdensome side-effects and long latency for efficacy with conventional agents, there is a continued need for generalised myasthenia gravis treatments that are safe and provide consistently sustained, long-term disease control. Nipocalimab, a neonatal Fc receptor blocker, was associated with dose-dependent reductions in total IgG and anti-acetylcholine receptor (AChR) antibodies and clinically meaningful improvements in the Myasthenia Gravis Activities of Daily Living (MG-ADL) scale in patients with generalised myasthenia gravis in a phase 2 study. We aimed to assess the safety and efficacy of nipocalimab in a phase 3 study.
View Article and Find Full Text PDFInt Immunopharmacol
January 2025
Health Science Center, Ningbo University, Ningbo, China. Electronic address:
Myasthenia gravis (MG) is a T cell-dependent, B cell-mediated disorder strongly associated with antigen presentation by dendritic cells (DCs). In MG, mucosal tolerance is linked to increased expression of TGF-β mRNA in monocytes. Additionally, monocytic myeloid-derived suppressor cells (M-MDSCs) exhibit negative immunomodulatory effects by suppressing autoreactive T and B cells.
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