These data are in agreement with previous data reported in two neuronal models of GCase deficiency following a two-week CBE treatment that led to degeneration linked to increased glucosylceramide and glucosylsphingosine, nuclear translocation of TFE3, LAMP1 upregulation, enhanced lysosome size, number and exocytosis37. Here, TFE3 is linked to hyperinsulinemic hypoglycemia, familial, 4.