Empagliflozin corrects protein glycosylation and oxidative burst in GSD-Ib patients after empagliflozin treatment. (A) Role for G6P (Gluc-6P) in uridine diphosphate glucose (UDP-Gluc) production (essential for protein glycosylation) and reduced NAD phosphate (NADPH) production (essential for the respiratory burst reactions). (B) Western blots illustrating the almost complete correction of glycosylation for the protein LAMP2 in granulocytes (PMNs) from PT1 and PT3 isolated before and during empagliflozin treatment and compared with a healthy control (CT). (C) Empagliflozin treatment corrected the defective respiratory burst in PT4. Whole blood was stimulated with phorbol myristate acetate (PMA) at 37°C for 15 minutes. Red blood cells were lysed, and the sample was analyzed immediately by flow cytometry with gating set on neutrophils. Gate B: neutrophils showing increased DHR123 fluorescence compared with background fluorescence in unstimulated cells. Gate C: PT4 PMA-stimulated neutrophils showing DHR123 fluorescence equivalent to that of the date-matched normal control. ADP, adenosine 5′-diphosphate; ATP, adenosine triphosphate; MW, molecular weight; PPi, inorganic pyrophosphate; UTP, G1P uridylyltransferase.