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Von gierke’s disease
• Von Gierke's disease is caused by a deficiency in
glucose 6-phosphatase, the enzyme that removes the
phosphoryl group from glucose and permits it to be
released from the cell (where it was presumably
stored as glycogen, or synthesized via
gluconeogenesis).
• Passage of the phosphorylated sugar through the cell
membrane is negligible; only the de-phosphorylated
form can be exported.
• Thus, when exercise raises energy demands on the
body, glycogen stores cannot be adequately mobilized
as glucose into the bloodstream to provide the
needed energy. 170
