Monoclonal antibodies against Torpedo acetylcholine receptor were used to define the binding regions of cross-reacting, anti-receptor antibodies from sera of myasthenic patients. Cross-reacting antibodies were directed mainly against the toxin-binding region of the receptor and a region remote from the acetylcholine-binding site. Few patients had antibodies against the acetylcholine-binding site region. The monoclonal antibodies provide probes for defining the heterogeneity of anti-receptor antibodies in myasthenic sera.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/0197-0186(82)90030-4 | DOI Listing |
Cureus
January 2025
Department of Neurology, Kobe City Medical Center General Hospital, Kobe, JPN.
Lambert-Eaton myasthenic syndrome (LEMS) is a paraneoplastic or autoimmune neuromuscular disorder that typically presents with limb weakness or autonomic dysfunction. Here, we report a rare case of LEMS with acute type 2 respiratory failure as the initial symptom. A 79-year-old woman was admitted with acute disturbance of consciousness and type 2 respiratory failure.
View Article and Find Full Text PDFNaunyn Schmiedebergs Arch Pharmacol
February 2025
Department of Neurology, First Affiliated Hospital, Chongqing Medical University, Chongqing, China.
Efgartigimod is a neonatal Fc receptor (FcRn) inhibitor that primarily diminishes antibody levels by inhibiting antibody (IgG) reabsorption and is noted for its rapid onset of action and low immunogenicity. Currently, efgartigimod is approved for the treatment of anti-acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (gMG), yet there are no relevant clinical trials for myasthenic crisis (MC). Consequently, we performed a systematic review of the available literature to evaluate the clinical efficacy of efgartigimod for the treatment of MC.
View Article and Find Full Text PDFActa Neuropathol Commun
February 2025
Department of Pediatric Neurology, Center for Neuromuscular Disorders in Children and Adolescents, University Hospital Essen, University Duisburg-Essen, Essen, Germany.
Mutations in CHRNE encoding the epsilon subunit of acetylcholine receptor result in impaired neuromuscular transmission and congenital myasthenic syndrome (CMS) with variying severity of symptoms. Although the pathophysiology is well-known, blood biomarker signatures enabling a patient-stratification are lacking. This retrospective two-center-study includes 19 recessive CHRNE-patients (AChR deficiency; mean age 14.
View Article and Find Full Text PDFFront Immunol
February 2025
Department of Neurology, Rambam Medical Center, Haifa, Israel.
Introduction: This case describes successful response to efgartigimod in the treatment of myasthenic crisis secondary to paraneoplastic disease, and in the perioperative setting.
Methods: An elderly female presented with speech difficulties, cessation of eating and 10kg weight loss over 4 months.
Results: Examination revealed ptosis, dysarthria, nasal speech, and weakness in limbs and neck flexors.
Int J Mol Sci
December 2024
Department of Neurology, Davis School of Medicine, University of California, 1515 Newton Court, Davis, CA 95618, USA.
We have designed and produced 39 amino acid peptide mimics of the and human acetylcholine receptors' (AChRs) main immunogenic regions (MIRs). These conformationally sensitive regions consist of three non-contiguous segments of the AChR α-subunits and are the target of 50-70% of the anti-AChR autoantibodies (Abs) in human myasthenic serum and in the serum of rats with a model of that disease, experimental autoimmune myasthenia gravis (EAMG), induced by immunizing the rats with the electric organ AChR. These MIR segments covalently joined together bind a significant fraction of the monoclonal antibodies (mAbs) raised in rats against electric organ AChR.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!