Autoantibodies; Autoimmunity; Myasthenia Gravis; Neuroimmunology
I am a scientist working in the field of myasthenia gravis and IgG4 autoimmunity. During my past research we have discovered the pathogenic mechanisms of MuSK IgG4 autoantibodies as a functional block of binding between MuSK and Lrp4, and demonstrated their pathogenicity in vitro. Then we discovered that IgG4 autoantibodies against MuSK are bi-specific in vivo, and that bispecific autoantibodies are pathogenic in vitro.My interest in IgG4 autoimmunity led to the development of a new classification system for IgG4 autoantibodies to determine the pathogenic role of IgG4 in autoimmune diseases.
Techniques, methods & infrastructure
Tissue- and cell-based assays as well as primary cell cultures are used to screen patient serum for the presence of autoantibodies against the neuromuscular junction. Immunoprecipitation and mass spectrometry are then used to identify the precise antigenic target of the antibodies. IgG subclass FACS is used to determine antigen-specific IgG subclasses.
- Biological Markers in Myasthenia gravis (2018)
Source of Funding: FWF (Austrian Science Fund), Hertha Firnberg Programme
- Koneczny, I., 2018. A New Classification System for IgG4 Autoantibodies. Frontiers in Immunology, 9. Available at: http://dx.doi.org/10.3389/fimmu.2018.00097.
- Koneczny, I. et al., 2017. IgG4 autoantibodies against muscle-specific kinase undergo Fab-arm exchange in myasthenia gravis patients. Journal of Autoimmunity, 77, pp.104-115. Available at: http://dx.doi.org/10.1016/j.jaut.2016.11.005.
- Koneczny, I. et al., 2019. Characterization of the thymus in Lrp4 myasthenia gravis: Four cases. Autoimmunity Reviews, 18(1), pp.50–55. Available at: http://dx.doi.org/10.1016/j.autrev.2018.07.011.
- Tomschik, M. et al., 2019. Severe Myasthenic Manifestation of Leptospirosis Associated with New Sequence Type of Leptospira interrogans. Emerging Infectious Diseases, 25(5), pp.968–971. Available at: http://dx.doi.org/10.3201/eid2505.181591.
- Koneczny, I. et al., 2013. MuSK Myasthenia Gravis IgG4 Disrupts the Interaction of LRP4 with MuSK but Both IgG4 and IgG1-3 Can Disperse Preformed Agrin-Independent AChR Clusters L. Mei, ed. PLoS ONE, 8(11), p.e80695. Available at: http://dx.doi.org/10.1371/journal.pone.0080695.