Cardiovascular progenitor cells and tissue plasticity are
reduced in a myocardium affected by Becker muscular dystrophy

J 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:

URL

Impact factor

Impact factor: 4.123

RIV identification code

RIV/00216224:14110/20:00115154

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1186/s13023-019-1257-4

UT WoS

000519036800001

Keywords in English

Becker muscular dystrophy; Dystrophin; Cardiovascular progenitor cells; C-kit; Cardiomyopathy; Heart failure

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

Name: CEITEC 2020 (Acronym: CEITEC2020)

Investor: Ministry of Education, Youth and Sports of the CR

ROZV/23/LF8/2019, interní kód MU

Name: Studium abnormalit lidských DMD kardiomyocytů

Investor: Ministry of Education, Youth and Sports of the CR, Internal development projects

ROZV/28/LF12/2020, interní kód MU

Name: Analýza arytmogenicity a kardiotoxicity vybraných plicních léčiv na kardiomyocytech diferencovaných z kmenových buněk

Investor: Ministry of Education, Youth and Sports of the CR, Internal development projects

7AMB13FR011, research and development project

Name: Přeprogramování somatických buněk darovaných pacienty s dědičnou Duchennovou svalovou dystrofií do kardiomyocytů - nahlédnutí do molekulární podstaty patologických dějů u dilatační kardiomyopatie nemocných DMD (Acronym: DUCHENSTEM)

Investor: Ministry of Education, Youth and Sports of the CR

Citovat

PEŠL, Martin, Šárka JELÍNKOVÁ, Guido CALUORI, Mária HOLICKÁ, Jan KREJČÍ, Petr NĚMEC, Aneta KOHUTOVÁ, Víta ŽAMPACHOVÁ, Petr DVOŘÁK and Vladimír ROTREKL. Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy. Orphanet Journal of Rare Diseases. London: England, 2020, vol. 15, No 1, p. 1-8. ISSN 1750-1172. Available from: https://dx.doi.org/10.1186/s13023-019-1257-4.

@article{1593819,
   author = {Pešl, Martin and Jelínková, Šárka and Caluori, Guido and Holická, Mária and Krejčí, Jan and Němec, Petr and Kohutová, Aneta and Žampachová, Víta and Dvořák, Petr and Rotrekl, Vladimír},
   article_location = {London},
   article_number = {1},
   doi = {http://dx.doi.org/10.1186/s13023-019-1257-4},
   keywords = {Becker muscular dystrophy; Dystrophin; Cardiovascular progenitor cells; C-kit; Cardiomyopathy; Heart failure},
   language = {eng},
   issn = {1750-1172},
   journal = {Orphanet Journal of Rare Diseases},
   title = {Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy},
   url = {https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-019-1257-4},
   volume = {15},
   year = {2020}
}

TY  - JOUR
ID  - 1593819
AU  - Pešl, Martin - Jelínková, Šárka - Caluori, Guido - Holická, Mária - Krejčí, Jan - Němec, Petr - Kohutová, Aneta - Žampachová, Víta - Dvořák, Petr - Rotrekl, Vladimír
PY  - 2020
TI  - Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy
JF  - Orphanet Journal of Rare Diseases
VL  - 15
IS  - 1
SP  - 1-8
EP  - 1-8
PB  - England
SN  - 17501172
KW  - Becker muscular dystrophy
KW  - Dystrophin
KW  - Cardiovascular progenitor cells
KW  - C-kit
KW  - Cardiomyopathy
KW  - Heart failure
UR  - https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-019-1257-4
L2  - https://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-019-1257-4
N2  - 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.
ER  -

PEŠL, Martin, Šárka JELÍNKOVÁ, Guido CALUORI, Mária HOLICKÁ, Jan KREJČÍ, Petr NĚMEC, Aneta KOHUTOVÁ, Víta ŽAMPACHOVÁ, Petr DVOŘÁK and Vladimír ROTREKL. Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy. \textit{Orphanet Journal of Rare Diseases}. London: England, 2020, vol.~15, No~1, p.~1-8. ISSN~1750-1172. Available from: https://dx.doi.org/10.1186/s13023-019-1257-4.

Detailed Information on Publication Record