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nitizinonas yra konkuruojantis 4 -hidroksifenilpiruvato dioksigenazs (fermento, esancio pries fumarilacetoacetato hidrolaz tirozino kataboliniame kelyje) inhibitorius.
nitisinone is a competitive inhibitor of 4-hydroxyphenylpyruvate dioxygenase, an enzyme which precedes fumarylacetoacetate hydrolase in the tyrosine catabolic pathway.
Last Update: 2008-03-04
Usage Frequency: 3
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slopindamas normal tirozino katabolizm serganci ht-1 pacient organizmuose, nitizinonas apsaugo nuo toksini tarpini jungini (maleiloacetoacetato ir fumarilacetoacetato) kaupimosi.
by inhibiting the normal catabolism of tyrosine in patients with ht-1, nitisinone prevents the accumulation of the toxic intermediates maleylacetoacetate and fumarylacetoacetate.
Last Update: 2008-03-04
Usage Frequency: 3
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biocheminis paveldimos 1 tipo (ht-1) tirozinemijos defektas yra fumarilacetoacetato hidrolazs (tirozino katabolinio kelio galutinio fermento) trkumas.
the biochemical defect in hereditary tyrosinemia type 1 (ht-1) is a deficiency of fumarylacetoacetate hydrolase, which is the final enzyme of the tyrosine catabolic pathway.
Last Update: 2008-03-04
Usage Frequency: 3
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