Modeling and rescue of defective blood–brain barrier function of induced brain microvascular endothelial cells from childhood cerebral adrenoleukodystrophy patients

Modeling and rescue of defective blood–brain barrier function of induced brain microvascular endothelial cells from childhood cerebral adrenoleukodystrophy patients

Abstract Background X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene. 40% of X-ALD patients will convert to the deadly childhood cerebral form (ccALD) characterized by increased permeability of the brain endothelium that constitutes the blood–brain barrier (BBB). Mutati...

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Bibliographic Details
Main Authors: Catherine A. A. Lee, Hannah S. Seo, Anibal G. Armien, Frank S. Bates, Jakub Tolar, Samira M. Azarin
Format: Article
Language:English
Published: BMC 2018-04-01
Series:Fluids and Barriers of the CNS
Subjects:
Adrenoleukodystrophy
In vitro human blood–brain barrier (BBB) model
Brain microvascular endothelial cells
Trans-endothelial electrical resistance
Human induced pluripotent stem cells (hiPSC)
Amphiphilic block copolymers
Online Access:http://link.springer.com/article/10.1186/s12987-018-0094-5

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