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...

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Bibliografiska uppgifter
Huvudupphovsmän:	Heare, T, Alp, N, Priestman, D, Kulkarni, AB, Qasba, P, Butters, T, Dwek, R, Clarke, K, Channon, K, Platt, F
Materialtyp:	Journal article
Språk:	English
Publicerad:	2007

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