Myasthenia gravis induced in mice by immunization with the recombinant extracellular domain of rat muscle-specific kinase (MuSK).
J Neuroimmunol
; 175(1-2): 107-17, 2006 Jun.
Article
em En
| MEDLINE
| ID: mdl-16697051
ABSTRACT
UNLABELLED Myasthenia gravis (MG) is mostly caused by anti-acetylcholine receptor (AChR) auto-antibodies (Abs). Such Abs are undetectable in 10-15% of MG patients, but many have anti-muscle-specific kinase (MuSK) Abs. We injected recombinant rat-MuSK extracellular domain in H-2(a), H-2(b), H-2(bm12) and H-2(d) mice. Certain strains exhibited exercise-induced fatigue, tremors, weight loss, and some died after 2-3 injections. Compound muscle action potentials showed decrement with low-frequency repetitive nerve stimulation. Miniature endplate potentials decreased, suggesting lower numbers of endplates functional AChRs. Myasthenic sera inhibited agrin-induced AChR aggregation in C2C12 myotubes. CONCLUSION:
Anti-MuSK Abs induce MG, which might also result from blocking the agrin-signaling pathway.
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Base de dados:
MEDLINE
Assunto principal:
Receptores Colinérgicos
/
Receptores Proteína Tirosina Quinases
/
Líquido Extracelular
/
Miastenia Gravis
Idioma:
En
Ano de publicação:
2006
Tipo de documento:
Article