Exploring the Correlation Between Fibrosis Biomarkers and Clinical Disease Severity in PLN p.Arg14del Patients.
| Autor: | van der Voorn SM; Division Heart and Lungs, Department of Medical Physiology, University Medical Center Utrecht, Utrecht, Netherlands., Bourfiss M; Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands., Te Riele ASJM; Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands., Taha K; Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands., Vos MA; Division Heart and Lungs, Department of Medical Physiology, University Medical Center Utrecht, Utrecht, Netherlands., de Brouwer R; Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands., Verstraelen TE; Heart Center, Department of Cardiology, Amsterdam University Medical Center, Location Academic Medical Center, Amsterdam, Netherlands., de Boer RA; Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands., Remme CA; Department of Clinical and Experimental Cardiology, Heart Centre, Amsterdam Univeristy Medical Center, Location Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands., van Veen TAB; Division Heart and Lungs, Department of Medical Physiology, University Medical Center Utrecht, Utrecht, Netherlands. |
|---|---|
| Jazyk: | angličtina |
| Zdroj: | Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2022 Jan 13; Vol. 8, pp. 802998. Date of Electronic Publication: 2022 Jan 13 (Print Publication: 2021). |
| DOI: | 10.3389/fcvm.2021.802998 |
| Abstrakt: | Background: Pathogenic variants in phospholamban ( PLN , like p. Arg14del), are found in patients diagnosed with arrhythmogenic (ACM) and dilated cardiomyopathy (DCM). Fibrosis formation in the heart is one of the hallmarks in PLN p.Arg14del carriers. During collagen synthesis and breakdown, propeptides are released into the circulation, such as procollagen type I carboxy-terminal propeptide (PICP) and C-terminal telopeptide collagen type I (ICTP). Aim: To investigate if PICP/ICTP levels in blood are correlative biomarkers for clinical disease severity and outcome in PLN p.Arg14del variant carriers. Methods: Serum and EDTA blood samples were collected from 72 PLN p.Arg14del carriers (age 50.5 years, 63% female) diagnosed with ACM ( n = 12), DCM ( n = 14), and preclinical variant carriers ( n = 46). PICP levels were measured with an enzyme-linked immune sorbent assay and ICTP with a radio immuno-assay. Increased PICP/ICTP ratios suggest a higher collagen deposition. Clinical data including electrocardiographic, and imaging results were adjudicated from medical records. Results: No correlation between PICP/ICTP ratios and late gadolinium enhancement (LGE) was found. Moderate correlations were found between the PICP/ICTP ratio and end-diastolic/systolic volume (both r Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 van der Voorn, Bourfiss, te Riele, Taha, Vos, de Brouwer, Verstraelen, de Boer, Remme and van Veen.) |
| Databáze: | MEDLINE |
| Externí odkaz: |
|