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Article

α-Galactosidase A knockout mice: progressive organ pathology resembles the type 2 later-onset phenotype of Fabry disease.

The American journal of pathology - 1 Mar 2015

Bangari Dinesh S, Ashe Karen M, Desnick Robert J, Maloney Colleen, Lydon John, Piepenhagen Peter, Budman Eva, Leonard John P, Cheng Seng H, Marshall John, Thurberg Beth L

Abstract excerpt

Fabry disease is an X-linked lysosomal storage disease caused by deficient activity of α-galactosidase A and the resultant systemic accumulation of globotrioasylceramide (GL-3) and related glycolipids. α-Galactosidase A gene knockout (Gla KO) mice have no α-galactosidase A activity and progressively accumulate GL-3 in tissues and fluids, similarly to FD patients. The nature and temporal effects of the progressive...

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PMID: 25553976
DOI: 10.1016/j.ajpath.2014.11.004

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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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