Klotho (-/-) mouse models have previously been used to study aging-related disease [23–25], and these models have shown that in these mice, phosphate and Ca2+ channel pathways (the sodium-phosphate cotransporters NaPi2a and TRPV5) are genetically altered, factors involved in the inflammation response (NF-κB, IL-6 and IL-8) are modulated, and oxidative stress signaling (ROS) is induced [26–28]. Here, KL is linked to glycogen storage disease VI.