Article ; Online: Laboratory Investigation of Hybrid IgG4 k/λ in MuSK Positive Myasthenia Gravis.
International journal of molecular sciences
2021 Volume 22, Issue 17
Abstract: ... resulting MuSK IgG4 k/λ Abs increase their interference on NMJ and pathogenicity. The characterization ... hybrid IgG4 k/λ, and the hybrid/total ratio in 14 MuSK-MG sera in comparison with 24 from MG with Abs ...
Abstract | Myasthenia gravis with antibodies (Abs) against the muscle-specific tyrosine kinase (MuSK) is a rare autoimmune disorder (AD) of the neuromuscular junction (NMJ) and represents a prototype of AD with proven IgG4-mediated pathogenicity. Thanks to the mechanism of Fab-arm exchange (FAE) occurring in vivo, resulting MuSK IgG4 k/λ Abs increase their interference on NMJ and pathogenicity. The characterization of hybrid MuSK IgG4 as a biomarker for MG management is poorly investigated. Here, we evaluated total IgG4, hybrid IgG4 k/λ, and the hybrid/total ratio in 14 MuSK-MG sera in comparison with 24 from MG with Abs against acetylcholine receptor (AChR) that represents the not IgG4-mediated MG form. In both subtypes of MG, we found that the hybrid/total ratio reflects distribution reported in normal individuals; instead, when we correlated the hybrid/total ratio with specific immune-reactivity we found a positive correlation only with anti-MuSK titer, with a progressive increase of hybrid/total mean values with increasing disease severity, indirectly confirming that most part of hybrid IgG4 molecules are engaged in the anti-MuSK pathogenetic immune-reactivity. Further analysis is necessary to strengthen the significance of this less unknown biomarker, but we retain it is full of a diagnostic-prognostic powerful potential for the management of MuSK-MG. |
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MeSH term(s) | Biomarkers/blood ; Humans ; Immunoglobulin G/blood ; Immunoglobulin G/immunology ; Myasthenia Gravis/blood ; Myasthenia Gravis/immunology ; Neuromuscular Junction/metabolism ; Neuromuscular Junction/pathology ; Receptor Protein-Tyrosine Kinases/immunology ; Receptors, Cholinergic/immunology |
Chemical Substances | Biomarkers ; Immunoglobulin G ; Receptors, Cholinergic ; MUSK protein, human (EC 2.7.10.1) ; Receptor Protein-Tyrosine Kinases (EC 2.7.10.1) |
Language | English |
Publishing date | 2021-08-24 |
Publishing country | Switzerland |
Document type | Journal Article |
ZDB-ID | 2019364-6 |
ISSN | 1422-0067 ; 1422-0067 ; 1661-6596 |
ISSN (online) | 1422-0067 |
ISSN | 1422-0067 ; 1661-6596 |
DOI | 10.3390/ijms22179142 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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