Table 1.

Mutations responsible for periodic paralysis (Venance et al., 2006; Jurkat-Rott et al., 2010; Statland and Barohn, 2013; Cannon, 2015; Sansone, 2019)

Type of periodic paralysisCausative ion channel mutationsType of mutation
Hyperkalemic periodic paralysis (hyperKPP) SCN4A (Nav1.4) Gain of function that increases NaP 
Hypokalemic periodic paralysis (hypoKPP) SCN4A (Nav1.4) and CACNA1S (Cav1.1) Gain of function that creates a gating pore current 
Andersen–Tawil syndrome KCNJ2 (Kir2.1) Loss of function that decreases the number of functional channels 
Type of periodic paralysisCausative ion channel mutationsType of mutation
Hyperkalemic periodic paralysis (hyperKPP) SCN4A (Nav1.4) Gain of function that increases NaP 
Hypokalemic periodic paralysis (hypoKPP) SCN4A (Nav1.4) and CACNA1S (Cav1.1) Gain of function that creates a gating pore current 
Andersen–Tawil syndrome KCNJ2 (Kir2.1) Loss of function that decreases the number of functional channels 

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