Thymoma related myasthenia gravis in humans and potential animal models

Marx, Alexander and Porubsky, Stefan and Belharazem, Djeda and Saruhan-Direskeneli, Guher and Schalke, Berthold and Stroebel, Philipp and Weis, Cleo-Aron (2015) Thymoma related myasthenia gravis in humans and potential animal models. EXPERIMENTAL NEUROLOGY, 270. pp. 55-65. ISSN 0014-4886, 1090-2430

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Abstract

Thymoma-associated Myasthenia gravis (TAMG) is one of the anti-acetylcholine receptor MG (AChR-MG) sub-types. The clinico-pathological features of TAMG and its pathogenesis are described here in comparison with pathogenetic models suggested for the more common non-thymoma AChR-MG subtypes, early onset MG and late onset MG. Emphasis is put on the role of abnormal intratumorous T cell selection and activation, lack of intratumorous myoid cells and regulatory T cells as well as deficient expression of the autoimmune regulator (AIRE) by neoplastic thymic epithelial cells. We review spontaneous and genetically engineered thymoma models in a spectrum of animals and the extensive clinical and immunological overlap between canine, feline and human TAMG. Finally, limitations and perspectives of the transplantation of human and murine thymoma tissue into nude mice, as potential models for TAMG, are addressed. (C) 2015 Elsevier Inc. All rights reserved.

Item Type: Article
Uncontrolled Keywords: ACETYLCHOLINE-RECEPTOR ANTIBODIES; THYMIC EPITHELIAL TUMORS; REGULATORY T-CELLS; PRAOMYS MASTOMYS NATALENSIS; BUF/MNA-RNU/+ RATS; EXFOLIATIVE DERMATITIS; MYOID CELLS; PROTEIN 4; CLASS-II; MUCOCUTANEOUS CANDIDIASIS; Thymoma; AIRE; Myasthenia gravis; APS-I; Animal model; Rat; Dog; Cat; Tiger; Horse; BUF/Mna
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Petra Gürster
Date Deposited: 21 Aug 2020 12:40
Last Modified: 21 Aug 2020 12:40
URI: https://pred.uni-regensburg.de/id/eprint/5163

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