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Article

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

Journal of inherited metabolic disease - 1 Feb 2007

Heare T, Alp N J, Priestman D A, Kulkarni A B, Qasba P, Butters T D, Dwek R A, Clarke K, Channon K M, Platt F M

Abstract excerpt

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 investigate aortic globotriaosylceramide levels, morphology and function in a mouse model of Fabry disease, and the...

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Source

Identifiers

PMID: 17189993
DOI: 10.1007/s10545-006-0473-y

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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