Therefore, the deficiency of aldolase B will end up with an accumulation of fructose-1-phosphate in liver cells, causing dysfunction and other sever problems (bioche). In addition to the toxicity of fructose-1-phosphate accumulation itself, excessive fructose-1-phosphate inhibits fructokinase, which contributes to fructosaemia (bioche). Another consequence of fructose-1-phosphate accumulation is the reduction of intracellular inorganic phosphate because inorganic phosphate is trapped in fructose-1-phosphate (HFI). An allosteric activation of adenosine deaminase triggered by the depleted inorganic phosphate concentration breaks down purine nucleotides to uric acid, and the accumulation of uric acid then causes hyperuricaemia (HFI). Another consequence of the depletion of inorganic phosphate is the ineffectiveness of phosphorylation in the glycogenolysis pathway (biochem). Inorganic phosphate is the substrate of hepatic glycogen phosphorylase (biochem). So when inorganic phosphate is deficient, hepatic glycogen phosphorylase cannot proceed phosphorylation normally, leading to an impaired glycogenolysis and eventually hypoglycaemia
Therefore, the deficiency of aldolase B will end up with an accumulation of fructose-1-phosphate in liver cells, causing dysfunction and other sever problems (bioche). In addition to the toxicity of fructose-1-phosphate accumulation itself, excessive fructose-1-phosphate inhibits fructokinase, which contributes to fructosaemia (bioche). Another consequence of fructose-1-phosphate accumulation is the reduction of intracellular inorganic phosphate because inorganic phosphate is trapped in fructose-1-phosphate (HFI). An allosteric activation of adenosine deaminase triggered by the depleted inorganic phosphate concentration breaks down purine nucleotides to uric acid, and the accumulation of uric acid then causes hyperuricaemia (HFI). Another consequence of the depletion of inorganic phosphate is the ineffectiveness of phosphorylation in the glycogenolysis pathway (biochem). Inorganic phosphate is the substrate of hepatic glycogen phosphorylase (biochem). So when inorganic phosphate is deficient, hepatic glycogen phosphorylase cannot proceed phosphorylation normally, leading to an impaired glycogenolysis and eventually hypoglycaemia