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Tyrosinemia type III is a rare disorder caused by a deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27), encoded by the gene HPD. [2] This enzyme is abundant in the liver, and smaller amounts are found in the kidneys. It is one of a series of enzymes needed to break down tyrosine.
[1] [2] Normally, the breakdown of the amino acid tyrosine involves the conversion of 4-hydroxyphenylpyruvate to homogentisate by 4-hydroxyphenylpyruvate dioxygenase. Complete deficiency of this enzyme would lead to tyrosinemia III. In rare cases, however, the enzyme is still able to produce the reactive intermediate 1,2-epoxyphenyl acetic acid ...
HPPD also catalyzes the conversion of phenylpyruvate to 2-hydroxyphenylacetate and the conversion of α-ketoisocaproate to β-hydroxy β-methylbutyrate. [2] [3] HPPD is an enzyme that is found in nearly all aerobic forms of life. [4] This reaction shows the conversion of 4-hydroxyphenylpyruvate into homogentisate by HPPD.
4-Hydroxyphenylpyruvic acid (4-HPPA) is an intermediate in the metabolism of the amino acid phenylalanine. The aromatic side chain of phenylalanine is hydroxylated by the enzyme phenylalanine hydroxylase to form tyrosine. The conversion from tyrosine to 4-HPPA is in turn catalyzed by tyrosine aminotransferase. [2]
L-tyrosine + 2-oxoglutarate 4-hydroxyphenylpyruvate + L-glutamate In humans, the tyrosine aminotransferase protein is encoded by the TAT gene . [ 7 ] A deficiency of the enzyme in humans can result in what is known as type II tyrosinemia , wherein there is an abundance of tyrosine as a result of tyrosine failing to undergo an aminotransferase ...
5. Eggs. You probably already know that eggs are a fantastic source of protein. They also happen to be packed with biotin, a vitamin often hyped for its nail-strengthening abilities. While ...
4-Hydroxyphenylpyruvate dioxygenase (HPPD) is an enzyme found in both plants and animals, which catalyzes the catabolism of the amino acid tyrosine. [4] Preventing the breakdown of tyrosine has three negative consequences: the excess of tyrosine stunts growth; the plant suffers oxidative damage due to lack of tocopherols (vitamin E); and ...
Don't rely on bloviating pundits to tell you who'll prevail on Hollywood's big night. The Huffington Post crunched the stats on every Oscar nominee of the past 30 years to produce a scientific metric for predicting the winners at the 2013 Academy Awards.