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Article

Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model.

Journal of lipid research - 1 Nov 2020

Jabbarzadeh-Tabrizi Siamak, Boutin Michel, Day Taniqua S, Taroua Mouna, Schiffmann Raphael, Auray-Blais Christiane, Shen Jin-Song

Abstract excerpt

Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), globotriaosylsphingosine (lyso-Gb3), and galabiosylceramide. However, there is no known link between these compounds and disease severity. In this study, we...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 32868283
DOI: 10.1194/jlr.RA120000909
PMCID: PMC7604726

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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