The role of mutations associated with familial neurodegenerative disorders on blood-brain barrier function in an iPSC model.

ABSTRACT:

Background

Blood-brain barrier dysfunction is associated with many late-stage neurodegenerative diseases. An emerging question is whether the mutations associated with neurodegenerative diseases can independently lead to blood-brain barrier (BBB) dysfunction. Studies from patient-derived induced pluripotent stem cells suggest that mutations associated with neurodegenerative disease are non-cell autonomous, resulting in gain of toxic function in derived neurons and astrocytes. Here we assess whether selected mutations associated with neurodegenerative diseases can contribute to impairment of the blood-brain barrier.

Methods

We assessed barrier function of confluent monolayers of human brain microvascular endothelial cells (hBMECs) derived from induced pluripotent stem cells (

SUBMITTER: Katt ME 

PROVIDER: S-EPMC6628493 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature