SUR1-mutant iPS cell-derived islets recapitulate the pathophysiology of congenital hyperinsulinism.

We have created a model recapitulating the known pathophysiology of KATPHI both in vitro and in vivo. We have also identified a novel role for KATP-channel activity during human islet development. This model will enable further studies for the improved understanding and clinical management of KATPHI without the need for primary patient tissue.