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Article

Endothelial Dysfunction in Fabry Disease Is Related to Glycocalyx Degradation.

Frontiers in immunology - 1 Jan 2021

Pollmann Solvey, Scharnetzki David, Manikowski Dominique, Lenders Malte, Brand Eva

Abstract excerpt

Fabry disease (FD) is an X-linked multisystemic lysosomal storage disease due to a deficiency of α-galactosidase A (GLA/AGAL). Progressive cellular accumulation of the AGAL substrate globotriaosylceramide (Gb3) leads to endothelial dysfunction. Here, we analyzed endothelial function in vivo and in vitro in an AGAL-deficient genetic background to identify the processes underlying this small vessel disease....

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Source

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Identifiers

PMID: 34917096
DOI: 10.3389/fimmu.2021.789142
PMCID: PMC8670230

Provenance

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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