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

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). Mutation infor...

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Detalles Bibliográficos
Autores principales: Lee, Catherine A. A., Seo, Hannah S., Armien, Anibal G., Bates, Frank S., Tolar, Jakub, Azarin, Samira M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5883398/
https://www.ncbi.nlm.nih.gov/pubmed/29615068
http://dx.doi.org/10.1186/s12987-018-0094-5