The human-severe combined immunodeficiency myasthenic mouse model: A new approach for the study of myasthenia gravis

G. Martino, B. L. DuPont, R. L. Wollmann, P. Bongioanni, J. Anastasi, J. Quintans, B. G W Arnason, L. M E Grimaldi

Research output: Contribution to journalArticle

Abstract

We have established a new chimeric human-mouse model of myasthenia gravis in severe combined immunodeficiency mice, using human peripheral blood lymphocytes that survive in the mouse and produce specific antibodies that mediate pathological changes typical of human myasthenia gravis. Mice given peripheral blood lymphocytes from both anti-acetylcholine receptor (AChR) antibody-positive and -negative patients with myasthenia gravis showed circulating anti-acetylcholine receptor antibodies, deposition of human IgG at muscle end-plates, and simplification of the postsynaptic membrane, findings characteristic of human myasthenia gravis. Mice given human peripheral blood lymphocytes from healthy donors and simultaneously immunized with Torpedo acetylcholine receptor showed the same changes. This chimeric model, utilizing human cells to reproduce the immunopathological findings of human myasthenia gravis in a nonhuman environment, offers new opportunities to study immune regulation in autoimmunity.

Original languageEnglish
Pages (from-to)48-56
Number of pages9
JournalAnnals of Neurology
Volume34
Issue number1
Publication statusPublished - 1993

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Severe Combined Immunodeficiency
Myasthenia Gravis
Muscle Weakness
Cholinergic Receptors
Lymphocytes
Antibodies
Torpedo
Autoimmunity
Immunoglobulin G
Tissue Donors
Muscles
Membranes

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Martino, G., DuPont, B. L., Wollmann, R. L., Bongioanni, P., Anastasi, J., Quintans, J., ... Grimaldi, L. M. E. (1993). The human-severe combined immunodeficiency myasthenic mouse model: A new approach for the study of myasthenia gravis. Annals of Neurology, 34(1), 48-56.

The human-severe combined immunodeficiency myasthenic mouse model : A new approach for the study of myasthenia gravis. / Martino, G.; DuPont, B. L.; Wollmann, R. L.; Bongioanni, P.; Anastasi, J.; Quintans, J.; Arnason, B. G W; Grimaldi, L. M E.

In: Annals of Neurology, Vol. 34, No. 1, 1993, p. 48-56.

Research output: Contribution to journalArticle

Martino, G, DuPont, BL, Wollmann, RL, Bongioanni, P, Anastasi, J, Quintans, J, Arnason, BGW & Grimaldi, LME 1993, 'The human-severe combined immunodeficiency myasthenic mouse model: A new approach for the study of myasthenia gravis', Annals of Neurology, vol. 34, no. 1, pp. 48-56.
Martino, G. ; DuPont, B. L. ; Wollmann, R. L. ; Bongioanni, P. ; Anastasi, J. ; Quintans, J. ; Arnason, B. G W ; Grimaldi, L. M E. / The human-severe combined immunodeficiency myasthenic mouse model : A new approach for the study of myasthenia gravis. In: Annals of Neurology. 1993 ; Vol. 34, No. 1. pp. 48-56.
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