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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.
The mechanism of action of nitisinone involves inhibition of 4-Hydroxyphenylpyruvate dioxygenase (HPPD). [5] [6] This is a treatment for patients with Tyrosinemia type 1 as it prevents the formation of 4-Maleylacetoacetic acid and fumarylacetoacetic acid, which have the potential to be converted to succinyl acetone, a toxin that damages the liver and kidneys. [4]
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 ...
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 ...
It is a 4-hydroxyphenylpyruvate dioxygenase inhibitor indicated for the treatment of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine. [7] Liver transplant is indicated for patients with tyrosinemia type I who do not respond to nitisinone, as well as those with acute liver failure and ...
6898 234724 Ensembl ENSG00000198650 ENSMUSG00000001670 UniProt P17735 Q8QZR1 RefSeq (mRNA) NM_000353 NM_146214 RefSeq (protein) NP_000344 NP_666326 Location (UCSC) Chr 16: 71.57 – 71.58 Mb Chr 8: 110.72 – 110.73 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Tyrosine aminotransferase (or tyrosine transaminase) is an enzyme present in the liver and catalyzes the conversion of ...
Mesotrione inhibits the enzyme 4-hydroxyphenylpyruvate dioxygenase (HPPD). [3] It is an extremely potent inhibitor of HPPD in laboratory tests using the plant Arabidopsis thaliana, with a K i value of about 10 pM. [6] In plants, HPPD is necessary for the biosynthesis of tocopherols and of plastoquinone, which is essential to carotenoid production.