Genetic disorders of aa breakdowns Flashcards
what can be supplied to the diet to treat urea enzyme deficiencies
benzoate and phenybutyrate
what do benzoate and phenybutyrate get converted to? what do their products do?
they get converted to benzoylglycine and phenylacetylglutamine
those products pull nitrogen out of the liver
what are the 2 major sources of NH4
dietary and recycled cellular proteins
whats special about the kidney and breakdown of amino acids into ammonia `
they get broken down into ammonia and then the ammonia can be transported right to the urine
where is free ammonia also produced from in the liver
serine and threonine can be broken into ammonia in liver
serine can make what in the liver
pyruvate and ammonia
threonine can make what in the liver
a-ketobutyrate and ammonia
what of the seven central metabolites are glucogenic
pyruvate
oxaloacetate
fumarate
what of the seven central metabolites are ketogenic
acetyl coa
acetoacetyl coa
what are the two amino acids that are purely ketogenic
leucine and lysine
what are the fates of branched amino acids
they get made into propionyl coa which gets made into succinylcholine coa which is an glycogenic precursor
what are the 3 branched amino acids
valine, leucine, isoleucine
where do branched chain amino acids get their nitrogen stripped? why can’t this happen in the liver
kidney, brain, and adipose
what enzyme breaks down the branched chain amino acids
branched chain a keto acid dehydrogenase complex
what amino acids are broken down to a-ketoglutarate
glutamine and glutamate
