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gaucher liga yra paveldimas metabolinis sutrikimas, atsirandantis nepavykus suskaidyti gliukozilceramid, o tai sukelia sios medziagos kaupimsi lizosomose ir vairiapusisk patologij.
gaucher disease is an inherited metabolic disorder caused by a failure to degrade glucosylceramide resulting in lysosomal storage of this material and widespread pathology.
最后更新: 2008-03-04
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veiklioji zavesca medziaga miglustatas nuslopina tam tikro fermento (gliukozilceramido sintazs) , paprastai gaminancio gliukozilceramid, veikl.
miglustat, the active substance in zavesca, prevents an enzyme (glucosylceramide synthase) from working.this enzyme normally produces glucosylceramide.
最后更新: 2008-03-04
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esant i tipo gošė (gaucher) ligai, medžiaga vadinama gliukozilceramidu, nepašalinama iš jūsų organizmo.
in type 1 gaucher disease, a substance called glucosylceramide is not removed from your body.
最后更新: 2017-04-26
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