Carl Schulz

Highlights • A concentration of unfiltered 1 μM RA is sufficient and necessary to induce a fully human-like atrial AP phenotype in hiPSC-CM • Sterile filtration of RA can lead to a relevant loss of RA preventing the induction of an adult atrial AP phenotype • APD90 shortening upon IKur block by 4-aminopyridine is present in human adult atrial tissue and atrial EHT • Sharp microelectrode measurements in intact tissues (EHT) remain the gold standard Summary Retinoic acid (RA) induces an atrial...

Highlights • Modeling of primary carnitine deficiency cardiomyopathy in hiPSC cardiomyocytes • Demonstration of low force and metabolic remodeling as key disease phenotype • Identification of ferroptosis and fibroblast activation as novel disease mechanisms Summary Primary carnitine deficiency (PCD) is an autosomal recessive monogenic disorder caused by mutations in SLC22A5. This gene encodes for OCTN2, which transports the essential metabolite carnitine into the cell.