Question

Patient's suffering from von Gierke's disease have a deficiency in the hydrolase that converts glucose-6-phosphate-glucose +inorganic phosphate (P). One of the most prominent symptoms of von Gierke's disease is a protruding abdomen due to an enlarged liver 3. a) How does the fate of glucose-6-phosphate in a von Gierke's patient explain the enlarged liver? b) Based on what you have learned so far, describe what would happen to a von Gierke's patient who has fasted?

Answer 1

von Gioke disease occu ed ove to o/o lark me glucose-6-phosphale hich is Usol to onve gbrose -6-phosphate he aánosmo/ of en to build up in cestain issves, Gkogenbseak е се maindy obses, v edin in in e ,sea se ),.ve is vo nhieske ally, the poos Aveing ih eis blood sloeam'İP he Rstwed, hence noma leveJr of olvcose in he blood s dec еос