compensatory activity of what enzyme takes over the role of fructose metabolism in essential fructosuriamore severe than essential fructosuria
hypoglycemia(because of the decreased available phosphate caused by fructose 1 phosphate accumulation)
fructose 1 phosphate cause jandice and cirrhosis
hyperammonemia
metabolic abnormalitiesornithine transcarbamylase deficiency(characterized by ↑orotic acid in blood and urine(part of the pyrimidine synthesis pathway))
medium-chain acyl-CoA dehydrogenase deficiency(MCAD deficiency)(fasting hypoglycemia! a bit like ethanol metabolism because of ↓NAD＋, other than the fact ↓acetyl CoA leads to no keton) metabolismbiochemistry HirotoShishido
maple syrup urine disease(MSUD)
deficient enzyme, symptomsbranched chain α-ketoacid dehydrogenase
→↑α-ketoacid in the blood(especially those from leucine)
