Hyperinsulinism of nfancy: the regulated release of insulin by K_ATP channel-independent pathways

摘要

Hyperinsulinism of infancy(HI)is a congenital defect in the regulated release of insulin from pancreatic β-cells. Here we describe stimulus-secretion coupling mechanisms in β-cells and intact islets of Langerhans isolated form three patients with a novel SURI gene defect. 2154±3 A to G SURI(GenBank accession num- ber L78207_is the first report of familial HI among nonconsanguineous Caucasians identified in the U.K. Using patch-clamp methodologies, we have shown that Number of operational ATP-sensitive K~+ channels(K_ATP Channels)inβ-cells and impaired ADP-depedendent reg- Ulation.