https://www.selleckchem.com/
Andersen-Tawil syndrome (ATS) is a very rare orphan genetic multisystem channelopathy without structural heart disease (with rare exceptions). ATS type 1 (ATS1) is inherited in an autosomal dominant fashion and is caused by mutations in the KCNJ2 gene, which encodes the α subunit of the K channel protein Kir2.1 (in ≈ 50 to 60% of cases). ATS type 2 (ATS2) is in turn linked to a rare mutation in the KCNJ5-GIRK4 gene that encodes the G protein-sensitive-activated inwardly rectifying K channel Kir3.4 (15%), which carries the K current IK(ACh). About 30% of cas