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nitisinone is een competitieve remmer van 4-hydroxyphenylpyruvaat dioxygenase, een enzym dat voorafgaat aan fumarylacetoacetaat hydrolase in de tyrosine katabole weg.
Чрез инхибиране на нормалния катаболизъм на тирозина при пациенти с ht- 1, нитисинонът
Last Update: 2011-10-23
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door het normale katabolisme van tyrosine in patiënten met ht-1 te remmen, voorkomt nitisinone de accumulatie van de toxische metabolieten maleylacetoacetaat en fumarylacetoacetaat.
4 предотвратява натрупването на токсичните междинни продукти малеилацетоацетат и фумарилацетоацетат.
Last Update: 2011-10-23
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het biochemisch defect in erfelijke tyrosinemie type 1 (ht-1) is een deficiëntie aan fumarylacetoacetaat hydrolyase, dat het eindenzym is van de tyrosine katabole weg.
Нитисинонът е конкурентен инхибитор на 4- хидроксифенилпируват диоксигеназа, един ензим, който предхожда фумарилацетоацетат хидролаза в катаболитния път на тирозина.
Last Update: 2012-04-11
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