Question

What is the role of substrate in producing a condition of HFI

Answer 1

Hereditary fructose intolerance (HFI) is caused due to lack of enzyme called aldolase B. This enzyme is required to process fructose.

A person with HFI on consumption of fructose shows lowering of blood sugar level (hypoglycemia) and accumulation of toxins in liver. This is because fructose due to lack of aldolase B results in formation of fructose-1-phosphate in cells especially in liver. This depletes inorganic phosphates and subsequently ATP synthesis in mitochondria of hepatocytes resulting in cell damage and glycogenolysis inhibition. In this way the substrate fructose contributes to HFI.