Modelling the Human Blood-Brain Barrier in Huntington Disease.

Autor: Vignone D; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Gonzalez Paz O; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Fini I; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Cellucci A; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Auciello G; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Battista MR; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Gloaguen I; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Fortuni S; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Cariulo C; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy., Khetarpal V; CHDI Management/CHDI Foundation, 6080 Center Drive, Los Angeles, CA 90045, USA., Dominguez C; CHDI Management/CHDI Foundation, 6080 Center Drive, Los Angeles, CA 90045, USA., Muñoz-Sanjuán I; CHDI Management/CHDI Foundation, 6080 Center Drive, Los Angeles, CA 90045, USA., Di Marco A; IRBM SpA, Via Pontina km 30,600, 00071 Pomezia, Italy.
Jazyk: angličtina
Zdroj: International journal of molecular sciences [Int J Mol Sci] 2022 Jul 15; Vol. 23 (14). Date of Electronic Publication: 2022 Jul 15.
DOI: 10.3390/ijms23147813
Abstrakt: 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.
Databáze: MEDLINE
Externí odkaz:
Nepřihlášeným uživatelům se plný text nezobrazuje