The human-severe combined immunodeficiency myasthenic mouse model: A new approach for the study of myasthenia gravis
| Autor: | Jose Quintans, Bonita L. DuPont, Barry G. W. Arnason, Gianvito Martino, Paolo Bongioanni, Luigi M.E. Grimaldi, Robert L. Wollmann, John Anastasi |
|---|---|
| Přispěvatelé: | Martino, Gianvito, Dupont, Bl, Wollmann, Rl, Bongioanni, P, Anastasi, J, Quintans, J, Arnason, Bgw, Grimaldi, Lme |
| Rok vydání: | 1993 |
| Předmět: |
Adult
Male Ratón Immunoglobulins Mice SCID medicine.disease_cause Motor Endplate Autoimmunity Mice Immunopathology Myasthenia Gravis medicine Animals Humans Receptors Cholinergic Receptor In Situ Hybridization Fluorescence Acetylcholine receptor Severe combined immunodeficiency biology medicine.disease Myasthenia gravis Disease Models Animal Neurology Lymphocyte Transfusion Immunology biology.protein Female Neurology (clinical) Antibody |
| Zdroj: | Annals of Neurology. 34:48-56 |
| ISSN: | 1531-8249 0364-5134 |
| DOI: | 10.1002/ana.410340110 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text