Thus, potassium supplementation rescues the expression of key molecules required for potassium homeostasis in RyR1AG/+ muscle, suggesting that KIR2.1, KATP6.1, KATP6.2, Prkaa1, Abbc9, and Na+, K+-ATPase α1 are potential biomarkers for myopathies associated with mutations in RyR1 and for evaluation of future potential therapies. Here, PRKAA1 is linked to myopathy.