Liver transplantation can cure some of these disorders. Energy is required to maneuver many amino acids from the intestinal tract into the blood or to reclaim them from the urine by special cells within the kidney. This transport of amino acids doesn't contain enzymes in metabolic pathways however fairly transport proteins embedded in cellular or intracellular organelle membranes. Mutant proteins with decreased transport actions might prevent the absorption of dietary amino acids or trigger their loss within the urine. For instance, in cystinuria there is elevated excretion of cystine, ornithine, arginine, and lysine in urine, which leads to kidney stones. Cystinosis is characterized by the defective egress of cystine out of cellular organelles called lysosomes owing to a defect within the transporter cystinosin
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Metabolic Disease Amino Acid, Disorders, Metabolism
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