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Title:Dynamics of leukocyte telomere length in patients with Fabry disease
Authors:ID Levstek, Tina (Author)
ID Breznik, Nika (Author)
ID Vujkovac, Bojan (Author)
ID Nowak, Albina (Author)
ID Trebušak Podkrajšek, Katarina (Author)
Files:.pdf PDF - Presentation file, download (731,45 KB)
MD5: A759E4D57021AA05463423E5FC00CA8F
 
URL URL - Source URL, visit https://www.mdpi.com/2227-9059/12/8/1724
 
Language:English
Typology:1.01 - Original Scientific Article
Organization:Logo UKC LJ - Ljubljana University Medical Centre
Abstract:Fabry disease (FD) leads to significant morbidity and mortality, which may indicate accelerated ageing. However, it is still unclear whether there is a relationship between telomere length (TL), a marker of biological ageing, and disease outcome. We aimed to examine the relationship between leukocyte TL (LTL) dynamics and the presence of advanced disease stages and/or late complications of FD, including hypertrophic cardiomyopathy, nephropathy and stroke, both cross-sectionally and longitudinally. DNA was extracted from peripheral blood leukocytes and quantitative PCR was utilized to determine relative LTL in 99 Fabry patients. In the longitudinal analysis, we included 50 patients in whom at least three measurements were performed over a period of 5–10 years. The results showed a significant inverse correlation between LTL and age (ρ = −0.20, p = 0.05). No significant differences in LTL were found between females and males (p = 0.79) or between patients receiving disease-specific therapy and those without (p = 0.34). In a cross-sectional analysis, no association was found between the presence (p = 0.15) or number (p = 0.28) of advanced stages of the disease and/or late complications and LTL. Similarly, in a longitudinal analysis, no difference in LTL dynamics was found regarding the presence (p = 0.16) of advanced stage organ involvement and/or late complications or their number. These findings indicate that LTL dynamics in adulthood may not be a reliable indicator of disease outcomes in Fabry patients. Therefore, LTL may more accurately reflect the disease burden in early life, when TL is primarily determined.
Keywords:Fabry disease, telomere length, hyperthropic cardiomyopathy, nephropathy, stroke
Publication status:Published
Publication version:Version of Record
Year of publishing:2024
Number of pages:str. 1-9
Numbering:Vol. 12, iss. 8, [article no.] 1724
PID:20.500.12556/DiRROS-29735 New window
UDC:616-07
ISSN on article:2227-9059
DOI:10.3390/biomedicines12081724 New window
COBISS.SI-ID:203575043 New window
Note: Nasl. z nasl. zaslona; Opis vira z dne 5. 8. 2024;
Publication date in DiRROS:04.06.2026
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Downloads:48
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Record is a part of a journal

Title:Biomedicines
Shortened title:Biomedicines
Publisher:MDPI AG
ISSN:2227-9059
COBISS.SI-ID:523006745 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:Fabrijeva bolezen, dolžina telomer, hipertrofična kardiomiopatija, nefropatija, kap


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