Fig. 3

Available models of MSA. a Toxin-induced models of MSA. The first models of MSA have been produced by injecting specific toxins (e.g. 6-hydroxydopamine, 3-nitropropionic acid, quinolinic acid and MPTP) into animals, thus inducing neurodegeneration of specific brain areas. These models are no longer considered as relevant disease models and have been substantially abandoned. b Animal models of MSA, obtained overexpressing human α-syn specifically in oligodendrocytes. The upper part of the panel shows transgenic mice which overexpress h-SNCA under the control of promoters of genes expressed in oligodendrocytes, namely myelin basic protein (MBP), proteolipid protein (PLP) or 2′,3′-cyclic nucleotide 3′-phosphodiesterase (CNP). The lower part of the panel shows a recently developed model obtained overexpressing human α-syn in oligodendrocytes of rodents and primates through viral vectors. c iPSC-based models of MSA. The expression of specific factors (Oct4, Sox2, Klf4 and c-Myc) allows to reprogram adult somatic cells, including fibroblasts and leukocytes, to induced pluripotent stem cells, which can then be differentiated toward all the different cellular subtypes of the organism, including neurons and glia