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Original Article
The large-conductance calcium-activated potassium channel holds the key to the conundrum of familial hypokalemic periodic paralysis
June-Bum Kim, Sung-Jo Kim, Sun-Yang Kang, Jin Woong Yi, Seung-Min Kim
Clin Exp Pediatr. 2014;57(10):445-450.   Published online October 31, 2014
Purpose

Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to improve the understanding...

Review Article
Channelopathies
June-Bum Kim
Clin Exp Pediatr. 2014;57(1):1-18.   Published online January 31, 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome,...