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ta encim sodeluje v prvi fazi izdelave glikosfingolipidov.
this enzyme is involved in the first step of the production of glycosphingolipids.
Son Güncelleme: 2017-04-26
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s preprečevanjem delovanja encima miglustat zmanjša tvorjenje glikosfingolipidov v celicah.
by preventing the enzyme from working, miglustat can reduce the production of glycosphingolipids in cells.
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nevrološke manifestacije se obravnavajo kot sekundarne na nenormalno kopičenje glikosfingolipidov v nevronalnih in glialnih celicah.
the neurological manifestations are considered secondary to the abnormal accumulation of glycosphingolipids in neuronal and glial cells.
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zdravilo zavesca deluje tako, da zavira delovanje encima, ki se imenuje 'glukozilceramid sintaza', ki je odgovoren za prvi korak sinteze večine glikosfingolipidov.
zavesca works by inhibiting the enzyme called ‘ glucosylceramide synthase’ which is responsible for the first step in the synthesis of most glycosphingolipids.
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Uyarı: Bu hizalama yanlış olabilir..
Böyle düşünüyorsanız lütfen silin.
fabryjeva bolezen je motnja skladiščenja glikosfingolipidov, ki jo povzroča premajhna aktivnost lizosomskega encima α-galaktozidaze a, kar povzroči kopičenje globotriaozilceramida (gb3 ali gl-3, imenovanega tudi ceramidtriheksozid (cth)), glikosfingolipidnega substrata tega encima.
fabry disease is a glycosphingolipid storage disorder that is caused by deficient activity of the lysosomal enzyme α-galactosidase a, resulting in accumulation of globotriaosylceramide (gb3 or gl-3, also known as ceramidetrihexoside (cth)), the glycosphingolipid substrate for this enzyme.
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