期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 23, 期 14, 页码 -出版社
MDPI
DOI: 10.3390/ijms23147813
关键词
blood-brain barrier; transport; induced pluripotent stem cells; brain endothelial cells; in vitro models; Huntington's disease
资金
- CHDI Foundation, Inc.
This study investigated the abnormalities and underlying mechanisms of blood-brain barrier (BBB) dysfunction in Huntington's disease using a model of human induced pluripotent stem cell-derived brain-like microvascular endothelial cells (iBMECs). The findings suggest that HD-iBMECs have abnormalities in barrier properties and specific BBB functions.
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 when promoting BBB dysfunction. Furthermore, the underlying mechanisms of BBB dysfunction remain elusive given the 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 demonstrated that HD-iBMECs have abnormalities in barrier properties, as well as in specific BBB functions such as receptor-mediated transcytosis.
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