Article

Decreased filamentous actin and tight junction protein expression, and paracellular permeability in Frataxin-deficient human brain microvascular endothelial cells – implications for blood-brain barrier integrity in Friedreich's Ataxia

2023-06-13

Abstract excerpt

<h4>Background: </h4> 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 progressive brain iron accumulation in the latter. End stage disease includes cardiac fibrosis that contribut...

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Literature Corpus work: d81b90fb-86ab-5107-927a-f4ece6fa646a
DOI: 10.21203/rs.3.rs-3025871/v1

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