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This aberrant GSL metabolism subsequently causes cellular dysfunction; however, the underlying cellular and molecular mechanisms are still unknown. There is growing evidence that damage to organelles, including lysosomes, mitochondria, and plasma membranes, is associated with substrate accumulation. Current methods for the detection of Gb3 are based on anti-Gb3 antibodies, the specificity and sensitivity of which are problematic for glycan detection. This study presents a robust method using lectins, specifically the B-subunit of Shiga toxin (StxB) from Shigella dysenteriae and LecA from Pseudomonas aeruginosa, as alternatives for Gb3 detection in Fabry fibroblasts by flow cytometry and confocal microscopy. StxB and LecA showed superior sensitivity, specificity, and consistency in different cell types compared to all anti-Gb3 antibodies used in this study. In addition, sphingolipid metabolism was analyzed in primary Fabry fibroblasts and \u03b1-Gal A knockout podocytes using targeted tandem liquid chromatography-mass spectrometry. Our findings establish lectins as a robust tool for improved diagnostics and research of Fabry disease and provide evidence of SL changes in cultured human cells, filling a knowledge gap.<\/jats:p>","DOI":"10.3390\/ijms26052272","type":"journal-article","created":{"date-parts":[[2025,3,4]],"date-time":"2025-03-04T03:26:21Z","timestamp":1741058781000},"page":"2272","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Lectin-Based Substrate Detection in Fabry Disease Using the Gb3-Binding Lectins StxB and LecA"],"prefix":"10.3390","volume":"26","author":[{"given":"Serap","family":"El\u00e7in-Guinot","sequence":"first","affiliation":[{"name":"Faculty of Biology, University of Freiburg, Sch\u00e4nzlestra\u00dfe 1, 79104 Freiburg, Germany"},{"name":"BIOSS, Centre for Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"CIBSS, Centre for Integrative Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5245-1781","authenticated-orcid":false,"given":"Simon","family":"Lagies","sequence":"additional","affiliation":[{"name":"Core Competence Metabolomics, Hilde-Mangold-Haus, University of Freiburg, Habsburgerstra\u00dfe 19, 79104 Freiburg, Germany"},{"name":"Institute of Organic Chemistry, University of Freiburg, Albertstra\u00dfe 19, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2020-4193","authenticated-orcid":false,"given":"Yoav","family":"Avi-Guy","sequence":"additional","affiliation":[{"name":"Faculty of Biology, University of Freiburg, Sch\u00e4nzlestra\u00dfe 1, 79104 Freiburg, Germany"},{"name":"BIOSS, Centre for Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"CIBSS, Centre for Integrative Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6209-3386","authenticated-orcid":false,"given":"Daniela","family":"Neugebauer","sequence":"additional","affiliation":[{"name":"Faculty of Biology, University of Freiburg, Sch\u00e4nzlestra\u00dfe 1, 79104 Freiburg, Germany"},{"name":"BIOSS, Centre for Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"CIBSS, Centre for Integrative Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tobias B.","family":"Huber","sequence":"additional","affiliation":[{"name":"III. Department of Medicine, University Medical Center Hamburg-Eppendorf (UKE), Martinistra\u00dfe 52, 20246 Hamburg, Germany"},{"name":"Hamburg Center for Kidney Health, University Medical Center Hamburg-Eppendorf (UKE), Martinistra\u00dfe 52, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christoph","family":"Schell","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Institute for Surgical Pathology Medical Center, University of Freiburg, Breisacher Str. 115A, 70106 Freiburg, Germany"},{"name":"Freiburg Institute for Advanced Studies (FRIAS), University of Freiburg, Albertstra\u00dfe 19, 79106 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernd","family":"Kammerer","sequence":"additional","affiliation":[{"name":"BIOSS, Centre for Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"Core Competence Metabolomics, Hilde-Mangold-Haus, University of Freiburg, Habsburgerstra\u00dfe 19, 79104 Freiburg, Germany"},{"name":"Institute of Organic Chemistry, University of Freiburg, Albertstra\u00dfe 19, 79104 Freiburg, Germany"},{"name":"Spemann Graduate School of Biology and Medicine (SGBM), University of Freiburg, Albertstra\u00dfe 19, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2847-246X","authenticated-orcid":false,"given":"Winfried","family":"R\u00f6mer","sequence":"additional","affiliation":[{"name":"Faculty of Biology, University of Freiburg, Sch\u00e4nzlestra\u00dfe 1, 79104 Freiburg, Germany"},{"name":"BIOSS, Centre for Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"CIBSS, Centre for Integrative Biological Signaling Studies, University of Freiburg, Sch\u00e4nzlestra\u00dfe 18, 79104 Freiburg, Germany"},{"name":"Freiburg Institute for Advanced Studies (FRIAS), University of Freiburg, Albertstra\u00dfe 19, 79106 Freiburg, Germany"},{"name":"Spemann Graduate School of Biology and Medicine (SGBM), University of Freiburg, Albertstra\u00dfe 19, 79104 Freiburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,3,4]]},"reference":[{"key":"ref_1","first-page":"2010","article-title":"Enzymatic defect in Fabry\u2019s disease. 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