August 8, 2026 · JACC. Clinical electrophysiology · DOI: 10.1016/j.jacep.2026.06.035

LMNA Deficiency Reveals a Role for SK3 Channel Dysfunction in Atrioventricular Block

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This study investigates the mechanisms underlying atrioventricular block (AVB) associated with the LMNA-R225X mutation, which affects nuclear envelope proteins and leads to cardiac dysfunction. The authors demonstrate that LMNA deficiency results in impaired function of the SK3 channel in pacemaker cells, contributing to AVB, and suggest that pharmacologic activation of the SK3 channel may serve as a novel therapeutic approach for managing this condition.

Tingting Li, Xiaolei Wang, Jia Song, Anna K Shinohara, Luge Li, Adeniyi Gbenga Adeleye, William Whitfield, Yaqueline Esperanza Gutierrez, Yue Yuan, Min Zhang, Bradley K McConnell, Na Li, Mihail G Chelu

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