Tyrosinemia

The tyrosine required by the human body is obtained by diet or by oxidizing phenylalanine. In addition to supplying synthetic protein, it is also a precursor of dopamine, adrenaline and melanin; excess tyrosine is degraded by it The pathway breaks down into carbon dioxide and water. Defective enzymes in each step in its metabolic pathway can cause a variety of diseases with different clinical manifestations. About 30% of premature babies and 10% of term newborns may develop temporary hypoxia due to liver 4-hydroxyphenylpyruvate dioxidase. Sexual hypertyrosineemia usually disappears within a few weeks after reducing the protein content of the diet to g / kg per day and vitamin C administration. Severe liver diseases often cause tyrosine metabolism disorders, which are caused by impaired activity of tyrosine transaminase, 4-hydroxyphenylpyruvate dioxidase, and urinate oxidase.