Question

can aldolase B deficiency affect glucogeonesis? and why?

Answer 1

Yes. Aldolase B deficiency can affected gluconeogenesis pathway.

Generally aldolase B present in liver cell and play an important role in Fructose metabolism. When fructose enter the cell then it's converted to Fructose 1-phosphate by the enzyme Fructokinase. Next aldolase B cleave Fructose 1-phosphate into Glyceraldehyde and dihydroxyacetone phosphate. And this two products undergoes either glycolytic or gluconeogenesis pathway.

Now if Aldolase B is absent or become nonfunctional then accumulation of Fructose 1-phosphate will occurs and also inorganic phosphate/free phosphate concentration in the cytosol will be decrease.Due to inorganic phosphate fall in cell and accumulation of fructose 1-phosphate results in Gluconeogenesis and glycolysis process become stopped. High amount of Fructose 1-phosphate also toxic for liver cell may cause cell death. Aldolase B deficiency thus cause Hereditary Fructose Intolerance(HFI).