mice and resulted in more improved motor functions, less aggregations in striatum and extended the lifespan of the animals (Yang and Yu, 2009). This further supports the importance of generating human and patient specific HD-iPS neuron cell models with endogenous CAG expansion to be used for cell replacement therapies as well as for drug screening and to enrich our knowledge in understanding mechanisms of HD (Table 2). The generation of efficient protocols for the differentiation of iPSCs into enriched populations of GABA MS-like neurons (GMSLNs) is indeed needed to provide a good model to investigate the disease manifestation and drug development (Nekrasov et al., 2016). HD-specific iPSCs were first generated in 2008 by Park et al. and they expressed an expanded CAG repeat sequences (72 repeats). The iPSCs were then differentiated into neural precursors by re-suspending colonies in EB differentiation medium in the absence of doxycycline with low-speed shaking (Park et al., 2008a).
