検索ワード: glikosfingolipidov (スロベニア語 - 英語)

ta encim sodeluje v prvi fazi izdelave glikosfingolipidov.
this enzyme is involved in the first step of the production of glycosphingolipids.

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.

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.

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.

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.