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...

Podrobná bibliografie
Hlavní autoři:	Heare, T, Alp, N, Priestman, D, Kulkarni, AB, Qasba, P, Butters, T, Dwek, R, Clarke, K, Channon, K, Platt, F
Médium:	Journal article
Jazyk:	English
Vydáno:	2007

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