Two human iPSC lines were employed in this study. Firstly, the CS83iCTR-33nXX (CTR33) line was used, which was derived from an unaffected sibling of a HD patient with genotyped CAG repeat length of 33 in the HTT gene. This line was reprogrammed using a non-integrating strategy and was developed as a “control” line for a related study on HD hiPSC characterization (Telezhkin et al., 2018 (link)). Secondly, the CS21iHD60n5 (HD60) line was used (Hd iPSC Consortium, 2017 (link)) which was derived from an HD juvenile patient and re-programmed using integrating vectors.
HiPSC lines were cultured and differentiated as previously described (Comella-Bolla et al., 2020 (link)). In brief, cells were kept in the pluripotent state using mTeSRTM1 (Stem Cell Technologies, Grenoble, France) on BD Matrigel-coated plates (BD Biosciences, Oxford, Oxon, United Kingdom), and differentiated to neural progenitors using an in-house differentiation protocol as described elsewhere (Comella-Bolla et al., 2020 (link)).
HiPSC lines were cultured and differentiated as previously described (Comella-Bolla et al., 2020 (link)). In brief, cells were kept in the pluripotent state using mTeSRTM1 (Stem Cell Technologies, Grenoble, France) on BD Matrigel-coated plates (BD Biosciences, Oxford, Oxon, United Kingdom), and differentiated to neural progenitors using an in-house differentiation protocol as described elsewhere (Comella-Bolla et al., 2020 (link)).
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