SREBP1 , a cholesterol biosynthesis regulator, was significantly upregulated in pluripotent SI-187 cells compared to one control and approached significance to the second control line; a finding consistent with overt cholesterol biosynthesis disruption in HD models (Valenza et al., 2007a , b ), patients (Markianos et al., 2008 ) and undifferentiated HD iPSC lines (Castiglioni et al., 2012 ).
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Characterization of forebrain neurons derived from late-onset Huntington's disease human embryonic stem cell lines.
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