Glycogen storage disease type I similar, but Ib incurs a few additional problems (described below). Glycogen in liver and (to a lesser degree) kidneys serves as a form of stored, rapidly accessible glucose, so that the blood glucose level can be maintained between meals. For about 3 hours after a carbohydrate-containing meal, high insulin levels direct liver cells to take glucose from the blood, to convert it to glucose-6-phosphate (G6P) with the enzyme glucokinase, and to add the G6P molecules to the ends of chains of glycogen (glycogen synthesis). Excess G6P is also shunted into production of triglycerides and exported for storage in adipose tissue
Adenosine monophosphate deaminase deficiency type 1 recycled back into glucose or burned by other cells in the body. However the newly generated glucose is made available to all cells in the body, not just to muscle cells. The ability of the body to assimilate lactate may also be diminished, if working muscle cells cannot take up glucose from blood, due to myophosphorylase maintaining a higher concentration of it inside loaded cells, and if liver has already filled its glycogen stores up to capacity. So, ultimately, in this state, working muscle cells are destined to lose all glycogen. AMP breakdown to adenosine in this state is minor,