Detailed Information on Publication Record
2020
Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy
PEŠL, Martin, Šárka JELÍNKOVÁ, Guido CALUORI, Mária HOLICKÁ, Jan KREJČÍ et. al.Basic information
Original name
Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy
Authors
PEŠL, Martin (203 Czech Republic, belonging to the institution), Šárka JELÍNKOVÁ (203 Czech Republic, belonging to the institution), Guido CALUORI (380 Italy, belonging to the institution), Mária HOLICKÁ (703 Slovakia), Jan KREJČÍ (203 Czech Republic, belonging to the institution), Petr NĚMEC (203 Czech Republic), Aneta KOHUTOVÁ (703 Slovakia, belonging to the institution), Víta ŽAMPACHOVÁ (203 Czech Republic, belonging to the institution), Petr DVOŘÁK (203 Czech Republic, belonging to the institution) and Vladimír ROTREKL (203 Czech Republic, guarantor, belonging to the institution)
Edition
Orphanet Journal of Rare Diseases, London, England, 2020, 1750-1172
Other information
Language
English
Type of outcome
Článek v odborném periodiku
Field of Study
30201 Cardiac and Cardiovascular systems
Country of publisher
United Kingdom of Great Britain and Northern Ireland
Confidentiality degree
není předmětem státního či obchodního tajemství
References:
Impact factor
Impact factor: 4.123
RIV identification code
RIV/00216224:14110/20:00115154
Organization unit
Faculty of Medicine
UT WoS
000519036800001
Keywords in English
Becker muscular dystrophy; Dystrophin; Cardiovascular progenitor cells; C-kit; Cardiomyopathy; Heart failure
Tags
International impact, Reviewed
Změněno: 7/2/2022 10:35, Mgr. Tereza Miškechová
Abstract
V originále
We describe the association of Becker muscular dystrophy (BMD) derived heart failure with the impairment of tissue homeostasis and remodeling capabilities of the affected heart tissue. We report that BMD heart failure is associated with a significantly decreased number of cardiovascular progenitor cells, reduced cardiac fibroblast migration, and ex vivo survival. Background Becker muscular dystrophy belongs to a class of genetically inherited dystrophin deficiencies. It affects male patients and results in progressive skeletal muscle degeneration and dilated cardiomyopathy leading to heart failure. It is a relatively mild form of dystrophin deficiency, which allows patients to be on a heart transplant list. In this unique situation, the explanted heart is a rare opportunity to study the degenerative process of dystrophin-deficient cardiac tissue. Heart tissue was excised, dissociated, and analyzed. The fractional content of c-kit(+)/CD45(-) cardiovascular progenitor cells (CVPCs) and cardiac fibroblast migration were compared to control samples of atrial tissue. Control tissue was obtained from the hearts of healthy organ donor's during heart transplantation procedures. Results We report significantly decreased CVPCs (c-kit(+)/CD45(-)) throughout the heart tissue of a BMD patient, and reduced numbers of phase-bright cells presenting c-kit positivity in the dystrophin-deficient cultured explants. In addition, ex vivo CVPCs survival and cardiac fibroblasts migration were significantly reduced, suggesting reduced homeostatic support and irreversible tissue remodeling. Conclusions Our findings associate genetically derived heart failure in a dystrophin-deficient patient with decreased c-kit(+)/CD45(-) CVPCs and their resilience, possibly hinting at a lack of cardioprotective capability and/or reduced homeostatic support. This also correlates with reduced plasticity of the explanted cardiac tissue, related to the process of irreversible remodeling in the BMD patient's heart.
Links
| LQ1601, research and development project |
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| ROZV/23/LF8/2019, interní kód MU |
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| ROZV/28/LF12/2020, interní kód MU |
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| 7AMB13FR011, research and development project |
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