{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T10:53:31Z","timestamp":1769597611372,"version":"3.49.0"},"reference-count":51,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,2,4]],"date-time":"2025-02-04T00:00:00Z","timestamp":1738627200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004329","name":"Slovenian Research Agency","doi-asserted-by":"publisher","award":["P1-0170"],"award-info":[{"award-number":["P1-0170"]}],"id":[{"id":"10.13039\/501100004329","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004329","name":"Slovenian Research Agency","doi-asserted-by":"publisher","award":["J3-50113"],"award-info":[{"award-number":["J3-50113"]}],"id":[{"id":"10.13039\/501100004329","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Takeda Pharmaceuticals","award":["P1-0170"],"award-info":[{"award-number":["P1-0170"]}]},{"name":"Takeda Pharmaceuticals","award":["J3-50113"],"award-info":[{"award-number":["J3-50113"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Fabry nephropathy is a life-threatening complication of Fabry disease characterized by complex and incompletely understood pathophysiological processes possibly linked to premature aging. We aimed to investigate leukocyte telomere length (LTL), oxidative stress, and kidney damage biomarkers in relation to kidney function. The study included 35 Fabry patients and 35 age and sex-matched control subjects. Based on the estimated slope of the glomerular filtration rate, the patients were divided into two groups. Relative LTL was quantified by qPCR, urinary biomarkers 8-hydroxy-2\u2032-deoxyguanosine (8-OHdG) and malondialdehyde (MDA) by UHPLC-MS\/MS, and kidney damage biomarkers by flow cytometry. There was no statistically significant difference in LTL between Fabry patients and controls. However, a significant difference was observed in male patients compared to their matched control subjects (p = 0.013). Oxidative stress biomarkers showed no differences between patients and controls, while significant differences were observed in urinary IGFBP7, EGF, and OPN levels between Fabry patients with stable kidney function and those with progressive nephropathy (FDR = 0.021, 0.002, and 0.013, respectively). Significant differences were also observed in plasma levels of cystatin C, TFF3, and uromodulin between patients with progressive nephropathy and controls (all FDR = 0.039). Along with these biomarkers (FDR = 0.007, 0.017, and 0.010, respectively), NGAL also exhibited a significant difference between the two patient groups (FDR = 0.017). This study indicates accelerated telomere attrition, which may be related to disease burden in males. Furthermore, analyses of urinary oxidative stress markers revealed no notable disparities between the different kidney function groups, indicating their limited utility. However, promising differences were found in some biomarkers of kidney damage in urine and plasma.<\/jats:p>","DOI":"10.3390\/cells14030218","type":"journal-article","created":{"date-parts":[[2025,2,4]],"date-time":"2025-02-04T05:13:43Z","timestamp":1738646023000},"page":"218","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Telomere Length, Oxidative Stress, and Kidney Damage Biomarkers in Fabry Nephropathy"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8808-1084","authenticated-orcid":false,"given":"Tina","family":"Levstek","sequence":"first","affiliation":[{"name":"Institute of Biochemistry and Molecular Genetics, Faculty of Medicine, University of Ljubljana, 1000 Ljubljana, Slovenia"},{"name":"Clinical Institute for Special Laboratory Diagnostics, University Children\u2019s Hospital, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erazem","family":"Bah\u010di\u010d","sequence":"additional","affiliation":[{"name":"Institute of Biochemistry and Molecular Genetics, Faculty of Medicine, University of Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5615-9606","authenticated-orcid":false,"given":"Bojan","family":"Vujkovac","sequence":"additional","affiliation":[{"name":"Centre for Fabry Disease, General Hospital Slovenj Gradec, 2380 Slovenj Gradec, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreja","family":"Cokan Vujkovac","sequence":"additional","affiliation":[{"name":"Centre for Fabry Disease, General Hospital Slovenj Gradec, 2380 Slovenj Gradec, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0234-2122","authenticated-orcid":false,"given":"Tine","family":"Tesovnik","sequence":"additional","affiliation":[{"name":"Clinical Institute for Special Laboratory Diagnostics, University Children\u2019s Hospital, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7650-6188","authenticated-orcid":false,"given":"\u017diga Iztok","family":"Remec","sequence":"additional","affiliation":[{"name":"Clinical Institute for Special Laboratory Diagnostics, University Children\u2019s Hospital, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanja","family":"\u010cuk","sequence":"additional","affiliation":[{"name":"Clinical Institute for Special Laboratory Diagnostics, University Children\u2019s Hospital, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0154-3547","authenticated-orcid":false,"given":"Katarina","family":"Trebu\u0161ak Podkraj\u0161ek","sequence":"additional","affiliation":[{"name":"Institute of Biochemistry and Molecular Genetics, Faculty of Medicine, University of Ljubljana, 1000 Ljubljana, Slovenia"},{"name":"Clinical Institute for Special Laboratory Diagnostics, University Children\u2019s Hospital, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1056\/NEJM196705252762101","article-title":"Enzymatic defect in Fabry\u2019s disease. 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