Aminotransferase Transamination Transaminase Pyridoxal phosphate Oxidative deamination L-glutamate dehydrogenase Glutamate synthetase Glucose-alanine cycle Ammonotelic Ureotelic Uricotelic Urea cycle Urea Creatine kinase Essential amino acids Ketogenic Glucogenic Tetrahydrofolate S-adenosylmethionine Phenylketonuria Mixed-function oxidase Alkaptonuria Maple syrup urine disease Most essential amino acids in biology glutamine glutamate aspartate and alanine
gastrin pepsinogen secretin cholecystokinin carboxypeptidase aminopepsidase PLP PLP is bond to the Lys residue and forms an internal aldimine when substrate binds, it becomes external aldimine form three different fates for oxidation deamination racemization decarboxylation L-glutarate dehydrogenase serves as the only enzyme to utilize two molecules, both NAD+ or NADP+ as substrate glutamate dehydrogenase L-glutamine the carrier of amino group glutamate dehydrogenase and glutaminase glucose alanine transport carbamoyl phosphate synthetase 1 mitochondria urea cycle carbamoyl phsophate synthetase 2 mitochondria, pyrimidine synthesis carbamoyl phosphate and ornithine citrulline citruyl AMP argininosuccinate fumarate and arginine ornithine ornithine transcarbamoylase ornithine from glutamate five enzyme in urea cycle Two ATP included in the formation of carbamoyl phosphate bicarbonate and ATP anhydride replacement ATP phosphorylation crbomylphosphate synthetase ornithine trancarbamoylase argininosuccinate synthetase argininosuccinase arginase malate aspartate shuttle aspartate argininosuccinate shunt malate alpha-ketoglutarate glutamate aspartate malate and aspartate 4 carbon glutamate and alpha-ketoglutarate 5 carbon aspartate aminotransferase aspartate aminotransferase glutamate oxaloacetate aminotransferase AAT and GOT creatine kinase carbamoyl phosphate synthetase 1 arginine surplus and N-acetylglutamate activate N-acetylglutamate synthase hyperammonemia