Transcriptome sequencing of human induced pluripotent stem cell-derived brain endothelial cells. Overall design: Examined one time point (Day 10 of differentiation) in triplicate from three different iPSC_BMECs. Sample name: iBMEC_33Q, iBMEC_71Q and iBMEC_109Q iBMEC: (iPSC)-derived brain-like microvascular endothelial cells Total samples: 9

While blood-brain barrier (BBB) dysfunction has been described in neurological disorders, including Huntington's disease (HD), it is not known if endothelial cells themselves are functionally compromised to promote BBB dysfunction. Further, the underlying mechanisms of BBB dysfunction remain elusive given limitations with mouse models and post-mortem tissue to identify primary deficits. We established models of BBB and undertook a transcriptome and functional analysis of human induced pluripotent stem cell (iPSC)-derived brain-like microvascular endothelial cells (iBMEC) from HD patients or unaffected controls. We demonstrate that HD-iBMECs have abnormalities in barrier properties, as well as in specific BBB functions such as receptor-mediated transcytosis.