Loss of filamentous actin, tight junction protein expression, and paracellular barrier integrity in frataxin-deficient human brain microvascular endothelial cells—implications for blood-brain barrier physiology in Friedreich’s ataxia

Loss of filamentous actin, tight junction protein expression, and paracellular barrier integrity in frataxin-deficient human brain microvascular endothelial cells—implications for blood-brain barrier physiology in Friedreich’s ataxia

Introduction: Friedreich’s Ataxia (FRDA) is the most prevalent inherited ataxia. FRDA results from loss of Frataxin (FXN), an essential mitochondrial iron trafficking protein. FRDA starts with an early burst of neurodegeneration of the dorsal root ganglion and cerebellar dentate nuclei, followed by...

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Bibliographic Details
Main Authors: Frances M. Smith, Daniel J. Kosman
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-01-01
Series:Frontiers in Molecular Biosciences
Subjects:
blood-brain barrier
permeability
filamentous actin
tight junction
transendothelial electrical resistance
frataxin
Online Access:https://www.frontiersin.org/articles/10.3389/fmolb.2023.1299201/full

Internet

https://www.frontiersin.org/articles/10.3389/fmolb.2023.1299201/full

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