Kidney histologic alterations in α-Galactosidase-deficient mice


Fabry disease is a rare X-linked disorder caused by mutations in the α-galactosidase gene (GLA), the resultant deficiency of lysosomal α-galactosidase enzyme activity leading to systemic accumulation of globotriaosylceramide and other glycosphingolipids. GLA knockout mice (“Fabry mice”) were generated as an animal model for Fabry disease but, as they do not… (More)
DOI: 10.1007/s00428-011-1051-8

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