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.Základní údaje
Originální název
Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy
Autoři
PEŠL, Martin (203 Česká republika, domácí), Šárka JELÍNKOVÁ (203 Česká republika, domácí), Guido CALUORI (380 Itálie, domácí), Mária HOLICKÁ (703 Slovensko), Jan KREJČÍ (203 Česká republika, domácí), Petr NĚMEC (203 Česká republika), Aneta KOHUTOVÁ (703 Slovensko, domácí), Víta ŽAMPACHOVÁ (203 Česká republika, domácí), Petr DVOŘÁK (203 Česká republika, domácí) a Vladimír ROTREKL (203 Česká republika, garant, domácí)
Vydání
Orphanet Journal of Rare Diseases, London, England, 2020, 1750-1172
Další údaje
Jazyk
angličtina
Typ výsledku
Článek v odborném periodiku
Obor
30201 Cardiac and Cardiovascular systems
Stát vydavatele
Velká Británie a Severní Irsko
Utajení
není předmětem státního či obchodního tajemství
Odkazy
Impakt faktor
Impact factor: 4.123
Kód RIV
RIV/00216224:14110/20:00115154
Organizační jednotka
Lékařská fakulta
UT WoS
000519036800001
Klíčová slova anglicky
Becker muscular dystrophy; Dystrophin; Cardiovascular progenitor cells; C-kit; Cardiomyopathy; Heart failure
Příznaky
Mezinárodní význam, Recenzováno
Změněno: 7. 2. 2022 10:35, Mgr. Tereza Miškechová
Anotace
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.
Návaznosti
| LQ1601, projekt VaV |
| ||
| ROZV/23/LF8/2019, interní kód MU |
| ||
| ROZV/28/LF12/2020, interní kód MU |
| ||
| 7AMB13FR011, projekt VaV |
|