Dystrophin Deficiency Causes Progressive Depletion of
Cardiovascular Progenitor Cells in the Heart

J 2021

Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart

JELÍNKOVÁ, Šárka, Yvonne SLEIMAN, Petr FOJTÍK, Franck AIMOND, Amanda FINAN et. al.

Basic information

Original name

Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart

Authors

JELÍNKOVÁ, Šárka (203 Czech Republic, belonging to the institution), Yvonne SLEIMAN, Petr FOJTÍK (203 Czech Republic, belonging to the institution), Franck AIMOND, Amanda FINAN, Gerald HUGON, Valerie SCHEUERMANN, Deborah BECKEROVÁ (203 Czech Republic, belonging to the institution), Olivier CAZORLA, Marie VINCENTI, Pascal AMEDRO, Sylvain RICHARD, Josef JAROŠ (203 Czech Republic, belonging to the institution), Petr DVOŘÁK (203 Czech Republic, belonging to the institution), Alain LACAMPAGNE, Gilles CARNAC, Vladimír ROTREKL (203 Czech Republic, guarantor, belonging to the institution) and Albano C. MELI

Edition

International Journal of Molecular Sciences, Basel, MDPI, 2021, 1422-0067

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

10608 Biochemistry and molecular biology

Country of publisher

Switzerland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 6.208

RIV identification code

RIV/00216224:14110/21:00124183

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.3390/ijms22095025

UT WoS

000650388200001

Keywords in English

duchenne muscular dystrophy; mdx mouse; cardiovascular progenitors; c-kit; genomic instability; dilated cardiomyopathy

Abstract

V originále

Duchenne muscular dystrophy (DMD) is a devastating condition shortening the lifespan of young men. DMD patients suffer from age-related dilated cardiomyopathy (DCM) that leads to heart failure. Several molecular mechanisms leading to cardiomyocyte death in DMD have been described. However, the pathological progression of DMD-associated DCM remains unclear. In skeletal muscle, a dramatic decrease in stem cells, so-called satellite cells, has been shown in DMD patients. Whether similar dysfunction occurs with cardiac muscle cardiovascular progenitor cells (CVPCs) in DMD remains to be explored. We hypothesized that the number of CVPCs decreases in the dystrophin-deficient heart with age and disease state, contributing to DCM progression. We used the dystrophin-deficient mouse model (mdx) to investigate age-dependent CVPC properties. Using quantitative PCR, flow cytometry, speckle tracking echocardiography, and immunofluorescence, we revealed that young mdx mice exhibit elevated CVPCs. We observed a rapid age-related CVPC depletion, coinciding with the progressive onset of cardiac dysfunction. Moreover, mdx CVPCs displayed increased DNA damage, suggesting impaired cardiac muscle homeostasis. Overall, our results identify the early recruitment of CVPCs in dystrophic hearts and their fast depletion with ageing. This latter depletion may participate in the fibrosis development and the acceleration onset of the cardiomyopathy.

Links

LQ1601, research and development project

Name: CEITEC 2020 (Acronym: CEITEC2020)

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

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

JELÍNKOVÁ, Šárka, Yvonne SLEIMAN, Petr FOJTÍK, Franck AIMOND, Amanda FINAN, Gerald HUGON, Valerie SCHEUERMANN, Deborah BECKEROVÁ, Olivier CAZORLA, Marie VINCENTI, Pascal AMEDRO, Sylvain RICHARD, Josef JAROŠ, Petr DVOŘÁK, Alain LACAMPAGNE, Gilles CARNAC, Vladimír ROTREKL and Albano C. MELI. Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart. International Journal of Molecular Sciences. Basel: MDPI, 2021, vol. 22, No 9, p. 1-20. ISSN 1422-0067. Available from: https://dx.doi.org/10.3390/ijms22095025.

@article{1834654,
   author = {Jelínková, Šárka and Sleiman, Yvonne and Fojtík, Petr and Aimond, Franck and Finan, Amanda and Hugon, Gerald and Scheuermann, Valerie and Beckerová, Deborah and Cazorla, Olivier and Vincenti, Marie and Amedro, Pascal and Richard, Sylvain and Jaroš, Josef and Dvořák, Petr and Lacampagne, Alain and Carnac, Gilles and Rotrekl, Vladimír and Meli, Albano C.},
   article_location = {Basel},
   article_number = {9},
   doi = {http://dx.doi.org/10.3390/ijms22095025},
   keywords = {duchenne muscular dystrophy; mdx mouse; cardiovascular progenitors; c-kit; genomic instability; dilated cardiomyopathy},
   language = {eng},
   issn = {1422-0067},
   journal = {International Journal of Molecular Sciences},
   title = {Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart},
   url = {https://www.mdpi.com/1422-0067/22/9/5025},
   volume = {22},
   year = {2021}
}

TY  - JOUR
ID  - 1834654
AU  - Jelínková, Šárka - Sleiman, Yvonne - Fojtík, Petr - Aimond, Franck - Finan, Amanda - Hugon, Gerald - Scheuermann, Valerie - Beckerová, Deborah - Cazorla, Olivier - Vincenti, Marie - Amedro, Pascal - Richard, Sylvain - Jaroš, Josef - Dvořák, Petr - Lacampagne, Alain - Carnac, Gilles - Rotrekl, Vladimír - Meli, Albano C.
PY  - 2021
TI  - Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart
JF  - International Journal of Molecular Sciences
VL  - 22
IS  - 9
SP  - 1-20
EP  - 1-20
PB  - MDPI
SN  - 14220067
KW  - duchenne muscular dystrophy
KW  - mdx mouse
KW  - cardiovascular progenitors
KW  - c-kit
KW  - genomic instability
KW  - dilated cardiomyopathy
UR  - https://www.mdpi.com/1422-0067/22/9/5025
N2  - Duchenne muscular dystrophy (DMD) is a devastating condition shortening the lifespan of young men. DMD patients suffer from age-related dilated cardiomyopathy (DCM) that leads to heart failure. Several molecular mechanisms leading to cardiomyocyte death in DMD have been described. However, the pathological progression of DMD-associated DCM remains unclear. In skeletal muscle, a dramatic decrease in stem cells, so-called satellite cells, has been shown in DMD patients. Whether similar dysfunction occurs with cardiac muscle cardiovascular progenitor cells (CVPCs) in DMD remains to be explored. We hypothesized that the number of CVPCs decreases in the dystrophin-deficient heart with age and disease state, contributing to DCM progression. We used the dystrophin-deficient mouse model (mdx) to investigate age-dependent CVPC properties. Using quantitative PCR, flow cytometry, speckle tracking echocardiography, and immunofluorescence, we revealed that young mdx mice exhibit elevated CVPCs. We observed a rapid age-related CVPC depletion, coinciding with the progressive onset of cardiac dysfunction. Moreover, mdx CVPCs displayed increased DNA damage, suggesting impaired cardiac muscle homeostasis. Overall, our results identify the early recruitment of CVPCs in dystrophic hearts and their fast depletion with ageing. This latter depletion may participate in the fibrosis development and the acceleration onset of the cardiomyopathy.
ER  -

JELÍNKOVÁ, Šárka, Yvonne SLEIMAN, Petr FOJTÍK, Franck AIMOND, Amanda FINAN, Gerald HUGON, Valerie SCHEUERMANN, Deborah BECKEROVÁ, Olivier CAZORLA, Marie VINCENTI, Pascal AMEDRO, Sylvain RICHARD, Josef JAROŠ, Petr DVOŘÁK, Alain LACAMPAGNE, Gilles CARNAC, Vladimír ROTREKL and Albano C. MELI. Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart. \textit{International Journal of Molecular Sciences}. Basel: MDPI, 2021, vol.~22, No~9, p.~1-20. ISSN~1422-0067. Available from: https://dx.doi.org/10.3390/ijms22095025.

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