Electrophysiology of human iPSC-derived vascular smooth muscle cells and cell autonomous consequences of Cantu Syndrome mutations.
Alex HansonConor McClenaghanKuo-Chan WengSarah ColijnAmber N StratmanCarmen M HalabiDorothy K GrangeJonathan R SilvaColin G NicholsPublished in: Function (Oxford, England) (2024)
The results show that hiPSC-VSMCs reiterate expression of the same major ion currents as primary VSMCs, validating the use of these cells to study vascular disease. Results in hiPSC-VSMCs derived from CS patient cells suggest that both the hypomyotonic and hyperelastic components of CS vasculopathy are cell-autonomous phenomena driven by KATP overactivity within VSMCs.