Abstract
Myotonic Dystrophy type 1 (DM1) is the most common form of muscular dystrophy in adults, characterized by a variety of multisystemic features and associated with cardiac anomalies. Among cardiac phenomena, conduction defects, ventricular arrhythmias, and dilated cardiomyopathy represent the main cause of sudden death in DM1 patients. Patient-specific induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) represent a powerful in vitro model for molecular, biochemical, and physiological studies of disease in the target cells. Here, we used an Atomic Force Microscope (AFM) to measure the beating profiles of a large number of cells, organized in CM clusters (Beating Bodies, BBs), obtained from wild type (WT) and DM1 patients. We monitored the evolution over time of the frequency and intensity of the beating. We determined the variations between different BBs and over various areas of a single BB, caused by morphological and biomechanical variations. We exploited the AFM tip to apply a controlled force over the BBs, to carefully assess the biomechanical reaction of the different cell clusters over time, both in terms of beating frequency and intensity. Our measurements demonstrated differences between the WT and DM1 cluste...Continue Reading
References
Apr 8, 1999·European Biophysics Journal : EBJ·J DomkeM Radmacher
Jun 19, 2001·American Journal of Physiology. Heart and Circulatory Physiology·H SauerM Wartenberg
Jul 14, 2004·IEEE Transactions on Bio-medical Engineering·Philip de ChazalRichard B Reilly
Feb 14, 2009·Circulation Research·Jianhua ZhangTimothy J Kamp
Apr 2, 2011·Biomedical Microdevices·Jose F Saenz CogolloRoberto Raiteri
Sep 6, 2011·American Journal of Physiology. Heart and Circulatory Physiology·Junyi MaCraig T January
Oct 27, 2011·Journal of Pharmacological Sciences·Masaki HondaTomoaki Inoue
May 25, 2012·PloS One·Jianwei LiuManish J Butte
Jun 19, 2012·Cell Stem Cell·Shinya Yamanaka
Dec 26, 2012·Analytical Chemistry·Wei-Tien ChangIan Liau
Dec 11, 2013·Stem Cells Translational Medicine·Ioannis KarakikesRoger J Hajjar
Dec 29, 2013·Journal of Molecular and Cellular Cardiology·Nikki H L van den HeuvelMalin K B Jonsson
Jul 20, 2014·Neurologic Clinics·Charles A Thornton
Feb 3, 2015·Nano Letters·Dario OssolaTomaso Zambelli
Dec 29, 2015·Biochimica Et Biophysica Acta·Eeva LaurilaKatriina Aalto-Setälä
Jan 8, 2016·Langmuir : the ACS Journal of Surfaces and Colloids·Tao YueMingjun Zhang
Mar 25, 2016·Nature Reviews. Cardiology·Ian Y ChenJoseph C Wu
Apr 27, 2016·World Journal of Stem Cells·Paola SpitalieriFederica Sangiuolo
Jun 9, 2016·Biosensors & Bioelectronics·Martin PeslVladimir Rotrekl
Nov 1, 2016·The Analyst·A T Chen, S Zou
Nov 18, 2016·Journal of Neuromuscular Diseases·Spandan KalraChris Denning
Dec 21, 2016·Journal of Molecular Recognition : JMR·Martin PeslVladimir Rotrekl
Jun 15, 2017·Circulation. Cardiovascular Genetics·Caroline Chong-NguyenDenis Duboc
Oct 27, 2017·Journal of Pharmacological and Toxicological Methods·Emanuela AbbateNejib Zemzemi