Anti-Ro52 antibody acts on the S5-pore linker of hERG to chronically reduce channel expression.

ABSTRACT:

Aims

The human ether-a-go-go-related gene (hERG) encodes the rapidly activating delayed rectifier potassium channel (IKr). Malfunction of hERG/IKr is the primary cause of acquired long QT syndrome (LQTS), an electrical disorder of the heart that can cause arrhythmias and sudden death. Patients with autoimmune diseases display a high incidence of LQTS. While dysfunction of hERG channels induced by autoantibodies such as anti-Ro52 may play a role in this pathology, the underlying mechanisms are not well understood. Here, we investigated the acute and chronic effects of anti-Ro52 antibody on hERG channels stably expressed in human embryonic kidney (hERG-HEK) 293 cells as well as IKr in neonatal rat ventricular myocytes.

Methods and results

Using whole-cell patch clamp, western

SUBMITTER: Szendrey J 

PROVIDER: S-EPMC6648346 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature