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...

詳細記述

書誌詳細
主要な著者: Heare, T, Alp, N, Priestman, D, Kulkarni, AB, Qasba, P, Butters, T, Dwek, R, Clarke, K, Channon, K, Platt, F
フォーマット: Journal article
言語:English
出版事項: 2007