Development of I<sub>KATP</sub> Ion Channel Blockers Targeting Sulfonylurea Resistant Mutant K<sub>IR</sub>6.2 Based Channels for Treating DEND Syndrome.
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ABSTRACT: Introduction: DEND syndrome is a rare channelopathy characterized by a combination of developmental delay, epilepsy and severe neonatal diabetes. Gain of function mutations in the KCNJ11 gene, encoding the KIR6.2 subunit of the IKATP potassium channel, stand at the basis of most forms of DEND syndrome. In a previous search for existing drugs with the potential of targeting Cantú Syndrome, also resulting from increased IKATP, we found a set of candidate drugs that may also possess the potential to target DEND syndrome. In the current work, we combined Molecular Modelling including Molecular Dynamics simulations, with single cell patch clamp electrophysiology, in order to test the effect of selected drug candidates on the KIR6.2 WT an
SUBMITTER: Houtman MJC
PROVIDER: S-EPMC8795863 | biostudies-literature | 2021
REPOSITORIES: biostudies-literature
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