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glikocerebrozīda uzkrāšanās aknās un liesā noved pie organomegālijas.
the accumulation of glucocerebroside in the liver and spleen leads to organomegaly.
Ultimo aggiornamento 2017-04-26
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imiglicerāze palīdz (katalizē) glikocerebrozīda sašķelšanu un novērš tā uzkrāšanos organismā.
imiglucerase helps (catalyses) the break down of glucocerebroside and stops it building up in the body.
Ultimo aggiornamento 2012-04-10
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Šī enzīma deficīts izraisa glikocerebrozīda uzkrāšanos, galvenokārt makrofāgos, kuri pārvēršas putu šūnās jeb “gošē šūnās”.
this enzymatic deficiency causes an accumulation of glucocerebroside primarily in macrophages, giving rise to foam cells or "gaucher cells".
Ultimo aggiornamento 2017-04-26
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alfa velaglicerāze papildina vai aizstāj bēta glikocerebrozidāzi – enzīmu, kurš lizosomā katalizē glikocerebrozīda hidrolīzi līdz glikozei un keramīdam, samazinot uzkrātā glikocerebrozīda daudzumu un koriģējot gošē slimības patofizioloģiju.
velaglucerase alfa supplements or replaces beta-glucocerebrosidase, the enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide in the lysosome, reducing the amount of accumulated glucocerebroside and correcting the pathophysiology of gaucher disease.
Ultimo aggiornamento 2017-04-26
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gošē slimībai ir raksturīga β- glikocerebrozidāzes enzimatīvās aktivitātes funkcionāla mazspēja, un tādēļ audu makrofāgos uzkrājas lipīds glikocerebrozīds, veidojot tā dēvētās gošē šūnas.
gaucher disease is characterised by a functional deficiency in β -glucocerebrosidase enzymatic activity and the resultant accumulation of lipid glucocerebroside in tissue macrophages, which become engorged and are termed gaucher cells.
Ultimo aggiornamento 2012-04-10
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