title: Thymoma related myasthenia gravis in humans and potential animal models.
abstract: Thymoma-associated Myasthenia gravis (TAMG) is one of the anti-acetylcholine receptor MG (AChR-MG) subtypes. 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.
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 13, D-68167 Mannheim, Germany. Electronic address: alexander.marx@umm.de.
authors.forename: Alexander
authors.initials: A
authors.lastname: Marx
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 13, D-68167 Mannheim, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Porubsky
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 13, D-68167 Mannheim, Germany.
authors.forename: Djeda
authors.initials: D
authors.lastname: Belharazem
authors.affiliation: Department of Physiology, Istanbul Faculty of Medicine, Istanbul University, Capa 34093, Istanbul, Turkey. Electronic address: gsaruhan@istanbul.edu.tr.
authors.forename: Güher
authors.initials: G
authors.lastname: Saruhan-Direskeneli
authors.affiliation: Department of Neurology, Bezirkskrankenhaus, University of Regensburg, D-93042 Regensburg, Germany. Electronic address: schalke@medbo.de.
authors.forename: Berthold
authors.initials: B
authors.lastname: Schalke
authors.affiliation: Institute of Pathology, University of Göttingen, Robert-Koch-Str. 40, D-37075 Göttingen, Germany. Electronic address: philipp.stroebel@med.uni-goettingen.de.
authors.forename: Philipp
authors.initials: P
authors.lastname: Ströbel
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 13, D-68167 Mannheim, Germany. Electronic address: cleo-aron.weis@medma.uni-heidelberg.de.
authors.forename: Cleo-Aron
authors.initials: CA
authors.lastname: Weis
mesh_terms: D000818:Animals; D002415:Cats; D004195:Disease Models, Animal; D004285:Dogs; D006801:Humans; D051379:Mice; D009157:Myasthenia Gravis / Q000150:complications / Q000276:immunology*; D013945:Thymoma / Q000150:complications / Q000276:immunology*; D013953:Thymus Neoplasms / Q000150:complications / Q000276:immunology*
keywords: AIRE; APS-I; Animal model; BUF/Mna; Cat; Dog; Horse; Myasthenia gravis; Rat; Thymoma; Tiger