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Title:Telomere length, oxidative stress, and kidney damage biomarkers in Fabry nephropathy
Authors:ID Levstek, Tina (Author)
ID Bahčič, Erazem (Author)
ID Vujkovac, Bojan (Author)
ID Cokan Vujkovac, Andreja (Author)
ID Tesovnik, Tine (Author)
ID Remec, Žiga Iztok (Author)
ID Čuk, Vanja (Author)
ID Trebušak Podkrajšek, Katarina (Author)
Files:.pdf PDF - Presentation file, download (1,53 MB)
MD5: A39139B615625DBE80FACD0EC4CAB68C
 
URL URL - Source URL, visit https://www.mdpi.com/2073-4409/14/3/218
 
Language:English
Typology:1.01 - Original Scientific Article
Organization:Logo UKC LJ - Ljubljana University Medical Centre
Abstract: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′ -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.
Keywords:nephropathy, telomere length, oxidative stress, kidney damage, biomarker, aging
Publication status:Published
Publication version:Version of Record
Year of publishing:2025
Number of pages:str. 1-15
Numbering:Vol. 14, no. 3
PID:20.500.12556/DiRROS-28878 New window
UDC:616-056.7
ISSN on article:2073-4409
DOI:10.3390/cells14030218 New window
COBISS.SI-ID:225070339 New window
Note:Nasl. z nasl. zaslona; Opis vira z dne 4. 2. 2025;
Publication date in DiRROS:10.04.2026
Views:126
Downloads:77
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Record is a part of a journal

Title:Cells
Shortened title:Cells
Publisher:MDPI
ISSN:2073-4409
COBISS.SI-ID:519958809 New window

Document is financed by a project

Funder:ARIS - Slovenian Research and Innovation Agency
Project number:P1-0170-2018
Name:Molekulski mehanizmi uravnavanja celičnih procesov v povezavi z nekaterimi boleznimi pri človeku

Funder:ARIS - Slovenian Research and Innovation Agency
Project number:J3-50113-2023
Name:Prepoznavanje in longitudinalna opredelitev bioloških označevalcev razvoja in napredovanja nefropatije pri Fabryjevi bolezni

Licences

License:CC BY 4.0, Creative Commons Attribution 4.0 International
Link:http://creativecommons.org/licenses/by/4.0/
Description:This is the standard Creative Commons license that gives others maximum freedom to do what they want with the work as long as they credit the author.

Secondary language

Language:Slovenian
Keywords:nefropatija, dolžina telomera, oksidativni stres, poškodbe ledvic, biomarker, staranje


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