| SVOBODOVÁ, Barbora, Šárka JELÍNKOVÁ, Martin PEŠL, Deborah BECKEROVÁ, Alain LACAMPAGNE, A. C. MELI and Vladimír ROTREKL. Cellular pathology of the human heart in Duchenne muscular dystrophy (DMD): lessons learned from in vitro modeling. PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY. NEW YORK: SPRINGER, 2021, vol. 473, No 7, p. 1099-1115. ISSN 0031-6768. Available from: https://dx.doi.org/10.1007/s00424-021-02589-0. |
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@article{1787537,
author = {Svobodová, Barbora and Jelínková, Šárka and Pešl, Martin and Beckerová, Deborah and Lacampagne, Alain and Meli, A. C. and Rotrekl, Vladimír},
article_location = {NEW YORK},
article_number = {7},
doi = {http://dx.doi.org/10.1007/s00424-021-02589-0},
keywords = {Cardiomyocyte; Contraction; Duchenne muscular dystrophy; In vitro modeling; Heart failure},
language = {eng},
issn = {0031-6768},
journal = {PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY},
title = {Cellular pathology of the human heart in Duchenne muscular dystrophy (DMD): lessons learned from in vitro modeling},
url = {https://link.springer.com/article/10.1007%2Fs00424-021-02589-0},
volume = {473},
year = {2021}
}
TY - JOUR
ID - 1787537
AU - Svobodová, Barbora - Jelínková, Šárka - Pešl, Martin - Beckerová, Deborah - Lacampagne, Alain - Meli, A. C. - Rotrekl, Vladimír
PY - 2021
TI - Cellular pathology of the human heart in Duchenne muscular dystrophy (DMD): lessons learned from in vitro modeling
JF - PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY
VL - 473
IS - 7
SP - 1099-1115
EP - 1099-1115
PB - SPRINGER
SN - 00316768
KW - Cardiomyocyte
KW - Contraction
KW - Duchenne muscular dystrophy
KW - In vitro modeling
KW - Heart failure
UR - https://link.springer.com/article/10.1007%2Fs00424-021-02589-0
N2 - Duchenne muscular dystrophy is a genetic disorder where an X-linked mutation in the DMD gene initiates pathogenic development caused by the absence of dystrophin protein. This impacts primarily the evolution of a functional muscle tissue resulting in muscle weakness and later severe disability in young male patients leading to an early death. Patients in the final stage develop dilated cardiomyopathy leading ultimately to cardiac or respiratory failure as the cause of death. This review discusses recent advances in modeling the DMD pathology in vitro. It describes in detail the molecular abnormalities found on the cellular and organoid levels. The in vitro pathology is compared to that found in patients. Likewise, the drawbacks and limitations of current models are discussed.
ER -
SVOBODOVÁ, Barbora, Šárka JELÍNKOVÁ, Martin PEŠL, Deborah BECKEROVÁ, Alain LACAMPAGNE, A. C. MELI and Vladimír ROTREKL. Cellular pathology of the human heart in Duchenne muscular dystrophy (DMD): lessons learned from in vitro modeling. \textit{PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY}. NEW YORK: SPRINGER, 2021, vol.~473, No~7, p.~1099-1115. ISSN~0031-6768. Available from: https://dx.doi.org/10.1007/s00424-021-02589-0.
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