As well as in another study on KCa3.1 functions performed in type-1 and -3 GD patients, we found a defect in the physiologically occurring upregulation of KCa3.1 during monocyte-to-macrophage differentiation [24, 25], suggesting that KCa3.1 dysregulation is a general feature of sphingolipidosis and cellular pathophysiology. This evidence concerns the gene KCNN4 and sphingolipidosis.