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cystathionine-bèta-synthase (cbs),
cystathionine beta-synthase (cbs),
Last Update: 2012-04-11
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n-acetylglutamaat synthase(nags)deficiëntie.
n-acetylglutamate synthase (nags) deficiency.
Last Update: 2017-04-26
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a16aa05 treatment of hyperammonaemia due to n-acetylglutamate synthase deficiency
a16aa05 treatment of hyperammonaemia due to n-acetylglutamate
Last Update: 2011-10-23
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wanneer de tegenovergestelde reactie belangrijker is bestaat de naam voor een deel uit synthase.
when the product is more important, synthase may be used in the name, e.g.
Last Update: 2016-03-03
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dat laatste gebeurt bijvoorbeeld in de ureum cyclus of bij de werking van de nitric oxide synthase enzymen.
citrullination is distinct from the formation of the free amino acid citrulline as part of the urea cycle or as a byproduct of enzymes of the nitric oxide synthase family.
Last Update: 2016-03-03
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*oligomycine zorgt ervoor dat het atp-synthase eiwit geen atp meer kan maken van de protonen.
as protons cross the membrane through the channel in the base of atp synthase, the fo proton-driven motor rotates.
Last Update: 2016-03-03
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het kan zijn dat meer dan 50% van de methionine synthase enzyme moleculen in het cytosol het inactieve cyanocobalamine bevatten.
it might quite well be that more than 50% of the methionine synthase enzyme molecules in the cytosol contains the inactive cyanocobalamin.
Last Update: 2016-03-03
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slechts 1% van het vrije cyanocobalamine wordt omgezet in de actieve vorm methylcobalamine, dat cofactor is in het methionine synthase enzyme.
only about 1% of free cyanocobalamin is converted to the active form methylcobalamin, which is cofactor in the methionine synthase enzyme.
Last Update: 2016-03-03
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het enzym cystathionine-bèta-synthase combineert serine en homocysteïne via een condensatiereactie tot de asymmetrische thio-ether cystathionine.
cystathionine beta-synthase then combines homocysteine and serine to form the asymmetrical thioether cystathionine.
Last Update: 2016-03-03
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dahp wordt dan omgezet tot 3-dehydrochinaat (dhq) met behulp van dhq-synthase en nad+ als cofactor.
dahp is then transformed to 3-dehydroquinate (dhq), in a reaction catalyzed by dhq synthase.
Last Update: 2016-03-03
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adjuvante behandeling van homocystinurie, waarbij tekorten of defecten zijn betrokken in: cystathionine-bèta-synthase (cbs),
adjunctive treatment of homocystinuria, involving deficiencies or defects in: cystathionine beta-synthase (cbs),
Last Update: 2017-04-26
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nitisinonebehandeling leidt tot genormaliseerd porfyrine metabolisme met normale erythrocyt pbg- synthase activiteit en urine 5-ala, verminderde urine uitscheiding van succinylaceton, verhoogde plasma tyrosineconcentratie en verhoogde urine uitscheiding van fenolzuren.
nitisinone treatment leads to normalised porphyrin metabolism with normal erythrocyte pbg- synthase activity and urine 5-ala, decreased urinary excretion of succinylacetone, increased plasma tyrosine concentration and increased urinary excretion of phenolic acids.
Last Update: 2011-10-23
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tijdens de aanvang van de therapie of als er een verslechtering optreedt, kan het echter naast bovenstaande tests nodig zijn om alle beschikbare biochemische parameters scherper te bewaken (d.w.z. plasma succinylaceton, urine 5-aminolevulinaat (ala) en erythrocyte porfobilinogeen (pbg)-synthase-activiteit).
however, in addition to the tests above, during the initiation of therapy or if there is a deterioration, it may be necessary to follow more closely all available biochemical parameters (i.e. plasma succinylacetone, urine 5-aminolevulinate (ala) and erythrocyte porphobilinogen (pbg)-synthase activity).
Last Update: 2017-04-26
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