Question

Patients with hereditary fructose intolerance are deficient in the liver form of the enzyme aldolase. Consumption of fructose leads to a depletion of ATP and \(P\), in the liver, which, in turn, leads to cell damage, Much of the cell damage can be attributed to the inability to maintain normal ion gradients by ATP-dependent pumps. The products initially produced by aldolase action on the substrate formed from fructose are A. two molecules of dihydroxyacetone phosphate. B. rwo molecules of glyceraldchyde 3 -phosphate. C. rwo molecules of lactate. D. dihydroxyacetone phosphate and glyceraldehyde 3 -phosphate. E. dihydroxyacctone phosphate and glyceraldehyde.

Short answer

Answer: Dihydroxyacetone phosphate and glyceraldehyde.

Step-by-step solution

Understand the process of fructose metabolism involving aldolase

In a healthy person, fructose is metabolized in the liver through a series of reactions to convert it into energy. The main enzyme involved in this process is Aldolase B, which cleaves the substrate fructose-1-phosphate into two products, dihydroxyacetone phosphate (DHAP) and glyceraldehyde. Then, glyceraldehyde is further phosphorylated to glyceraldehyde-3-phosphate by the action of Triose kinase.

Analyze the effect of deficient aldolase in HFI patients

In patients with hereditary fructose intolerance, the enzyme aldolase B is deficient. As a result, the reaction that converts fructose-1-phosphate into DHAP and glyceraldehyde slows down. This causes an accumulation of fructose-1-phosphate in the liver. This can lead to a series of metabolic problems.

Identify the products initially formed by aldolase

Considering that aldolase B enzyme acts on the substrate formed from fructose (fructose-1-phosphate), the products initially formed by its action are dihydroxyacetone phosphate (DHAP) and glyceraldehyde. The enzymatic action will look like this: Fructose-1-phosphate (substrate) --(Aldolase B)--> Dihydroxyacetone phosphate + Glyceraldehyde

Choose the correct option

Based on the analysis in steps 1-3, the correct option among the given alternatives is: D. dihydroxyacetone phosphate and glyceraldehyde 3 -phosphate. Note: There is a typo in option D, as it should be "dihydroxyacetone phosphate and glyceraldehyde" (without "3-phosphate" in the glyceraldehyde).