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Frataxin-deficient human brain microvascular endothelial cells lose polymerized actin and are paracellularly permeable –implications for blood-brain barrier integrity in Friedreich’s Ataxia

BACKGROUND: Friedreich’s Ataxia (FRDA) is the most prevalent inherited ataxia; the disease results from loss of Frataxin, an essential mitochondrial iron trafficking protein. FRDA presents as neurodegeneration of the dorsal root ganglion and cerebellar dentate nuclei, followed by brain iron accumula...

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Detalles Bibliográficos
Autores principales:	Smith, Frances M., Kosman, Daniel J
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Cold Spring Harbor Laboratory 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9934603/ https://www.ncbi.nlm.nih.gov/pubmed/36798283 http://dx.doi.org/10.1101/2023.02.09.527936

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