Final answer:
GSD I is linked to a deficiency in the enzyme glucose-6-phosphate dehydrogenase (G6PD), which can cause premature breakdown of red blood cells and result in anemia.
Step-by-step explanation:
Children with the condition known as GSD I are unable to release glucose from glycogen stored in the liver due to a deficiency in the enzyme glucose-6-phosphate dehydrogenase (G6PD). G6PD deficiency is an X-linked recessive hereditary disease that leads to lower than normal levels of the G6PD enzyme.
This enzyme is vital as it is the rate-limiting step in the pentose phosphate pathway, which is particularly important for maintaining levels of NADPH in cells. Since NADPH is critical for maintaining the level of glutathione, which protects red blood cells against oxidative damage, a deficiency can cause red blood cells to break down prematurely leading to anemia, a common consequence of G6PD deficiency.