title: Multiple sclerosis animal models: a clinical and histopathological perspective.
abstract: There is a broad consensus that multiple sclerosis (MS) represents more than an inflammatory disease: it harbors several characteristic aspects of a classical neurodegenerative disorder, that is, damage to axons, synapses and nerve cell bodies. While we are equipped with appropriate therapeutic options to prevent immune-cell driven relapses, effective therapeutic options to prevent the progressing neurodegeneration are still missing. In this review article, we will discuss to what extent pathology of the progressive disease stage can be modeled in MS animal models. While acute and relapsing-remitting forms of experimental autoimmune encephalomyelitis (EAE), which are T cell dependent, are aptly suited to model relapsing-remitting phases of MS, other EAE models, especially the secondary progressive EAE stage in Biozzi ABH mice is better representing the secondary progressive phase of MS, which is refractory to many immune therapies. Besides EAE, the cuprizone model is rapidly gaining popularity to study the formation and progression of demyelinating CNS lesions without T cell involvement. Here, we discuss these two non-popular MS models. It is our aim to point out the pathological hallmarks of MS, and discuss which pathological aspects of the disease can be best studied in the various animal models available.
authors: Department of Neuroanatomy, Faculty of Medicine, LMU München University, München, 80336, Germany.
authors: Markus
authors: M
authors: Kipp
authors: Department of Neuroanatomy, Faculty of Medicine, LMU München University, München, 80336, Germany.|Institute of Neuroanatomy, Faculty of Medicine, RWTH Aachen University, Aachen, D-52074, Germany.
authors: Stella
authors: S
authors: Nyamoya
authors: Department of Neuroanatomy, Faculty of Medicine, LMU München University, München, 80336, Germany.
authors: Tanja
authors: T
authors: Hochstrasser
authors: Department of Pathology, VU University Medical Centre, Amsterdam, The Netherlands.|Barts and The London School of Medicine and Dentistry, Neuroimmunology Unit, , Queen Mary University of London, Neuroscience Centre, Blizard Institute of Cell and Molecular Science, London, UK.
authors: Sandra
authors: S
authors: Amor
mesh_terms: D000818:Animals; D001369:Axons / Q000473:pathology; D003471:Cuprizone / Q000494:pharmacology; D003711:Demyelinating Diseases / Q000473:pathology; D004195:Disease Models, Animal; D018450:Disease Progression; D004681:Encephalomyelitis, Autoimmune, Experimental / Q000473:pathology; D006801:Humans; D056784:Leukoencephalopathies / Q000473:pathology; D009103:Multiple Sclerosis / Q000473:pathology* / Q000628:therapy; D019636:Neurodegenerative Diseases / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology
chemical_list: D003471:Cuprizone
keywords: EAE; PPMS; SPMS; cuprizone; disability; disease progression; multiple sclerosis; neurodegeneration; treatment