Patients With Long-QT Syndrome Caused by Impaired hERG-Encoded K_v11.1 Potassium Channel Have Exaggerated Endocrine Pancreatic and Incretin Function Associated With Reactive Hypoglycemia.

ABSTRACT:

Background

Loss-of-function mutations in hERG (encoding the K_v11.1 voltage-gated potassium channel) cause long-QT syndrome type 2 (LQT2) because of prolonged cardiac repolarization. However, K_v11.1 is also present in pancreatic α and β cells and intestinal L and K cells, secreting glucagon, insulin, and the incretins glucagon-like peptide-1 (GLP-1) and GIP (glucose-dependent insulinotropic polypeptide), respectively. These hormones are crucial for glucose regulation, and long-QT syndrome may cause disturbed glucose regulation. We measured secretion of these hormones and cardiac repolarization in response to glucose ingestion in LQT2 patients with functional mutations in hERG and matched healthy participants, testing the hypothesis that LQT2 patie

SUBMITTER: Hylten-Cavallius L 

PROVIDER: S-EPMC5412733 | biostudies-literature | 2017 May

REPOSITORIES: biostudies-literature