Similar to GAMT and AGAT mice, AMPK activation, as measured by phospho-AMPK (pAMPK) or phosphorylated substrate (pACC) levels, was increased in CT1-/y mice indicating a chronic catabolic state, possibly resulting from energy deficiency, in skeletal muscle (Figure 6F). Here, SLC6A8 is linked to hyperinsulinemic hypoglycemia, familial, 4.