| JELÍNKOVÁ, Šárka, Aneta KOHUTOVÁ, Petr FOJTÍK, Miriama KRUTÁ, Aleksandra VILOTIĆ, Martin PEŠL, Jan VRBSKÝ, Renata GAILLYOVÁ, Iveta VALÁŠKOVÁ, Ivan FRÁK, Giancarlo FORTE, Petr DVOŘÁK, Albano MELI, Vladimír ROTREKL, Lenka MARKOVÁ, Tereza JURÁKOVÁ and Alain LACAMPAGNE. Dystrophin Deficiency Leads to Genomic Instability in Human Pluripotent Stem Cells via NO Synthase-Induced Oxidative Stress. Cells. Basel: MDPI, 2019, vol. 8, No 1, p. 1-22. ISSN 2073-4409. Available from: https://dx.doi.org/10.3390/cells8010053. |
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@article{1498116,
author = {Jelínková, Šárka and Kohutová, Aneta and Fojtík, Petr and Krutá, Miriama and Vilotić, Aleksandra and Pešl, Martin and Vrbský, Jan and Gaillyová, Renata and Valášková, Iveta and Frák, Ivan and Forte, Giancarlo and Dvořák, Petr and Meli, Albano and Rotrekl, Vladimír and Marková, Lenka and Juráková, Tereza and Lacampagne, Alain},
article_location = {Basel},
article_number = {1},
doi = {http://dx.doi.org/10.3390/cells8010053},
keywords = {DMD; dystrophin; pluripotent stem cells; genome stability; ROS; NO synthases},
language = {eng},
issn = {2073-4409},
journal = {Cells},
title = {Dystrophin Deficiency Leads to Genomic Instability in Human Pluripotent Stem Cells via NO Synthase-Induced Oxidative Stress},
url = {http://dx.doi.org/10.3390/cells8010053},
volume = {8},
year = {2019}
}
TY - JOUR
ID - 1498116
AU - Jelínková, Šárka - Kohutová, Aneta - Fojtík, Petr - Krutá, Miriama - Vilotić, Aleksandra - Pešl, Martin - Vrbský, Jan - Gaillyová, Renata - Valášková, Iveta - Frák, Ivan - Forte, Giancarlo - Dvořák, Petr - Meli, Albano - Rotrekl, Vladimír - Marková, Lenka - Juráková, Tereza - Lacampagne, Alain
PY - 2019
TI - Dystrophin Deficiency Leads to Genomic Instability in Human Pluripotent Stem Cells via NO Synthase-Induced Oxidative Stress
JF - Cells
VL - 8
IS - 1
SP - 1-22
EP - 1-22
PB - MDPI
SN - 20734409
KW - DMD
KW - dystrophin
KW - pluripotent stem cells
KW - genome stability
KW - ROS
KW - NO synthases
UR - http://dx.doi.org/10.3390/cells8010053
L2 - http://dx.doi.org/10.3390/cells8010053
N2 - Recent data on Duchenne muscular dystrophy (DMD) show myocyte progenitor’s involvement in the disease pathology often leading to the DMD patient’s death. The molecular mechanism underlying stem cell impairment in DMD has not been described. We created dystrophin-deficient human pluripotent stem cell (hPSC) lines by reprogramming cells from two DMD patients, and also by introducing dystrophin mutation into human embryonic stem cells via CRISPR/Cas9. While dystrophin is expressed in healthy hPSC, its deficiency in DMD hPSC lines induces the release of reactive oxygen species (ROS) through dysregulated activity of all three isoforms of nitric oxide synthase (further abrev. as, NOS). NOS-induced ROS release leads to DNA damage and genomic instability in DMD hPSC. We were able to reduce both the ROS release as well as DNA damage to the level of wild-type hPSC by inhibiting NOS activity.
ER -
JELÍNKOVÁ, Šárka, Aneta KOHUTOVÁ, Petr FOJTÍK, Miriama KRUTÁ, Aleksandra VILOTI$\backslash$'C, Martin PEŠL, Jan VRBSKÝ, Renata GAILLYOVÁ, Iveta VALÁŠKOVÁ, Ivan FRÁK, Giancarlo FORTE, Petr DVOŘÁK, Albano MELI, Vladimír ROTREKL, Lenka MARKOVÁ, Tereza JURÁKOVÁ and Alain LACAMPAGNE. Dystrophin Deficiency Leads to Genomic Instability in Human Pluripotent Stem Cells via NO Synthase-Induced Oxidative Stress. \textit{Cells}. Basel: MDPI, 2019, vol.~8, No~1, p.~1-22. ISSN~2073-4409. Available from: https://dx.doi.org/10.3390/cells8010053.
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