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
hereditary fructose intolerance
symptoms
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