Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment.

Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment.

OBJECTIVE: Fabry disease results from alpha-gala-ctosidase A deficiency and is characterized by the lysosomal accumulation of globotriaosylceramide. Globotriaosylceramide storage predominantly affects endothelial cells, altering vascular wall morphology and vasomotor function. Our objective was to i...

Bibliografiset tiedot
Päätekijät:	Heare, T, Alp, N, Priestman, D, Kulkarni, AB, Qasba, P, Butters, T, Dwek, R, Clarke, K, Channon, K, Platt, F
Aineistotyyppi:	Journal article
Kieli:	English
Julkaistu:	2007

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Correction: Globotriaosylceramide Accumulation and Not Alpha-Galactosidase-A Deficiency Causes Endothelial Dysfunction in Fabry Disease
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