In order to investigate the impact of stomatin deficiency in RBCs on the activity of AE1, a stopped-flow assay based, as previously described31, on the measurements of the kinetics of pHi changes related to HCO3− entry was applied to ghosts derived from four OHSt and one 7.2(−)CHC patients. Here, SLC4A1 is linked to hyperinsulinemic hypoglycemia, familial, 4.