ABCD1 dysfunction alters white matter microvascular perfusion.
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ABSTRACT:
SUBMITTER: Lauer A
PROVIDER: S-EPMC5841142 | biostudies-literature | 2017 Dec
REPOSITORIES: biostudies-literature
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ABCD1 dysfunction alters white matter microvascular perfusion.
Lauer Arne A Da Xiao X Hansen Mikkel Bo MB Boulouis Gregoire G Ou Yangming Y Cai Xuezhu X Liberato Celso Pedrotti Afonso A Kalpathy-Cramer Jayashree J Caruso Paul P Hayden Douglas L DL Rost Natalia N Mouridsen Kim K Eichler Florian S FS Rosen Bruce B Musolino Patricia L PL
Brain : a journal of neurology 20171201 12
Cerebral X-linked adrenoleukodystrophy is a devastating neurodegenerative disorder caused by mutations in the ABCD1 gene, which lead to a rapidly progressive cerebral inflammatory demyelination in up to 60% of affected males. Selective brain endothelial dysfunction and increased permeability of the blood-brain barrier suggest that white matter microvascular dysfunction contributes to the conversion to cerebral disease. Applying a vascular model to conventional dynamic susceptibility contrast mag ...[more]
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