In contrast to G6PC1 deficiency, which causes hypoglycemia, lactic acidosis and accumulation of glycogen in liver and in kidney (Glycogen Storage Disease type Ia), G6PC3 deficiency does not cause metabolic symptoms, but it is consistently associated with a severe neutropenia and, at least in humans, with heart, blood vessel and urogenital tract malformations.18, 19 Both G6PC1 and G6PC3 are trans‐membrane proteins that are inserted in the endoplasmic reticulum membrane with their catalytic site oriented towards the lumen of this organelle. This evidence concerns the gene G6PC3 and lactic acidosis.