Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity

Maartje G. Huijbers*, Anna Fleur D. Vink, Erik H. Niks, Ruben H. Westhuis, Erik W. van Zwet, Robert H. de Meel, Ricardo Rojas García, Jordi Díaz Manera, Jan B. Kuks, Rinse Klooster, Kirsten Straasheijm, Amelia Evoli, Isabel Illa, Silvère M. van der Maarel, Jan J. Verschuuren

*Autore corrispondente per questo lavoro

Risultato della ricerca: Contributo in rivistaArticolopeer review

30 Citazioni (Scopus)

Abstract

Muscle weakness in MuSK myasthenia gravis (MG) is caused predominantly by IgG4 antibodies which block MuSK signalling and destabilize neuromuscular junctions. We determined whether the binding pattern of MuSK IgG4 antibodies change throughout the disease course ("epitope spreading"), and affect disease severity or treatment responsiveness.We mapped the MuSK epitopes of 255 longitudinal serum samples of 53 unique MuSK MG patients from three independent cohorts with ELISA.Antibodies against the MuSK Iglike-1 domain determine disease severity. Epitope spreading outside this domain did not contribute to disease severity nor to pyridostigmine responsiveness. This provides a rationale for epitope specific treatment strategies.
Lingua originaleInglese
pagine (da-a)82-88
Numero di pagine7
RivistaJournal of Neuroimmunology
Volume291
Numero di pubblicazioneFebbraio
DOI
Stato di pubblicazionePubblicato - 2016
Pubblicato esternamente

All Science Journal Classification (ASJC) codes

  • Immunologia e Allergia
  • Immunologia
  • Neurologia
  • Neurologia (clinica)

Keywords

  • 80 and over
  • Adult
  • Aged
  • Autoantibodies
  • Cholinergic
  • Cholinesterase Inhibitors
  • Enzyme-Linked Immunosorbent Assay
  • Epitope Mapping
  • Epitope mapping
  • Female
  • Humans
  • IgG4
  • Immunology
  • Immunology and Allergy
  • Italy
  • Longitudinal Studies
  • Male
  • Middle Aged
  • MuSK
  • Myasthenia Gravis
  • Myasthenia gravis
  • Neurology
  • Neurology (clinical)
  • Neuromuscular junction
  • Receptor Protein-Tyrosine Kinases
  • Receptors
  • Severity of Illness Index
  • Spain
  • Statistics as Topic
  • Young Adult

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