Thanks to the transformation of fibroblasts collected from the skin of adult patients suffering from the so-called long QT syndrome – a deadly disturbance of the heart rhythm, the stem cells and muscle cells of the heart can be carefully examined, understood and treated, according to scientists from Israel in the journal Nature.
Long QT syndrome (LQTS) is a congenital or acquired disorder characterized by paroxysmal ventricular tachycardia and sudden cardiac death. The QT interval on the ECG is a graphical representation of the depolarization and repolarization of the ventricles. In a properly functioning heart, the length of the QT interval shortens as the heart rate increases. QT prolongation promotes the onset of life-threatening ventricular arrhythmias: ventricular fibrillation and polymorphic ventricular tachycardia.
Lior Gepstein, together with colleagues from the Israel Institute of Technology in Haifa, cultured heart muscle cells (cardiomyocytes) from connective tissue cells (fibroblasts) taken from the skin of adult patients with long QT syndrome. Fibroblasts were first transformed into undifferentiated stem cells and then plated under conditions inducing cardiomyocyte differentiation. The resulting heart muscle cells had abnormal electrical activity characteristic of LQTS disease.
After growing cardiomyocytes with disorders, scientists treated them with various drugs and checked how it influenced the electrophysiological activity of cells. As they emphasize, thanks to their work, it will be possible to test new drugs and check their effects in terms of treatment of a specific patient. (PAP)