Vous avez cherché: glucosylceramide (Anglais - Maltais)

in gaucher disease glucosylceramide levels can get too high.
fil-każ tal- marda gaucher il-livelli tal-glucosylceramide jistgħu jkunu għoljin aktar milli suppost.

this enzyme helps the body control levels of glucosylceramide.
din l-enżima tgħin lill-ġisem jikkontrolla l-livelli tal-glucosylceramide.

glucosylceramide is a natural substance in the body, made of sugar and fat.
glucosylceramide hi sustanza naturali fil-ġisem, magħmula minn zokkor u xaħam.

as a result glucosylceramide builds up in your spleen, liver and bones.
b’konsegwenza ta’ dan, glucosylceramide jakkumula fil- milsa, fil-fwied jew fl-għadam tiegħek.

in type 1 gaucher disease, a substance called glucosylceramide is not removed from your body.
fil-marda ta’ gaucher ta’ tip 1, sustanza msejħa glucosylceramide ma titneħħiex minn ġismek.

in vitro, glucosylceramide synthase is inhibited by miglustat with an ic50 of 20-37 µm.
in vitro, glucosylceramide synthase huwa inibit minn miglustat b’ ic50 ta ’ 20- 37 µm.

the inhibitory action on glucosylceramide synthase forms the rationale for substrate reduction therapy in gaucher disease.
l-azzjoni ta’ inibizzjoni fuq glucosylceramide synthase hi r-raġuni għat-terapija tat-tnaqqis tas-sottostrat (substrate reduction therapy) fil-marda ta’ gaucher.

the active substance in zavesca, miglustat, prevents an enzyme called glucosylceramide synthase from working.
is-sustanza attiva f’zavesca, il-miglustast, tipprevjeni enzima msejjħa sintażi tal-glukosilċeramide ) milli taħdem.

miglustat is an inhibitor of glucosylceramide synthase, the enzyme responsible for the first step in the synthesis of most glycolipids.
miglustat hu impeditur ta’ glycosylceramide synthase, l-enzima responsabbli għall-ewwel pass tas-sintesi ta’ ħafna mill-glycolipids.

imiglucerase replaces the missing enzyme in gaucher disease, helping to break down glucosylceramide and stopping it building up in the body.
imiglucerase tissostitwixxi l- enzima nieqsa fil-marda gaucher, biex b’hekk tgħin fit-tkissir ta’ glucosylceramide u twaqqafha milli takkumula fil-ġisem.

gaucher disease is an inherited metabolic disorder caused by a failure to degrade glucosylceramide resulting in lysosomal storage of this material and widespread pathology.
il-marda ta’ gaucher hi marda metabolika li tintiret ġenetikament li hi kkawżata min-nuqqas fil-kapaċità tal-ġisem biex ikisser il-glucosylceramide, li jirriżulta fil-ħżin ta’ lysosomal ta’ dan il-materjal u f’patoloġija mifruxa.

the active substance in cerdelga, eliglustat, works by blocking the action of an enzyme involved in the production of the glucosylceramide fat.
is-sustanza attiva f’cerdelga, eliglustat, taħdem billi timblokka l-azzjoni tal-enzima li hija involuta fil- produzzjoni tax-xaħam glukosilċeramide.

eliglustat is a potent and specific inhibitor of glucosylceramide synthase, and acts as a substrate reduction therapy (srt) for gd1.
eliglustat huwa inibitur qawwi u speċifiku ta’ glucosylceramide synthase, u jaġixxi bħala terapija li tnaqqas is-substrat (substrate reduction therapy, srt) għal gd1.

zavesca works by inhibiting the enzyme called ‘glucosylceramide synthase’ which is responsible for the first step in the synthesis of most glycosphingolipids.
zavesca jaħdem billi jinibixxi l-enzima msejjħa ‘glucosylceramide synthase’ li hija responsabbli għall- ewwel pass fis-sintesi tal-biċċa l-kbira tal-glycosphingolipids.

patients with this disease lack an enzyme called glucocerebrosidase, resulting in a glycosphingolipid called glucosylceramide building up in different parts of the body, such as the spleen, liver and bones.
pazjenti b’ din il- marda għandhom nuqqas ta ’ enzim imsajjaħ glukoċerebrosidażi,, li jwassal għal żieda fil- livell ta ’ sustanza glukosilċeramide f’ partijiet differenti tal- ġisem, bħall- milsa, il- fwied u l- għadam.

patients with this disease lack an enzyme called glucocerebrosidase, which results in a glycosphingolipid called glucosylceramide building up in different parts of the body, such as the spleen, liver and bones.
pazjenti b’din il- marda għandhom nuqqas ta’ enzimi msejħa glukoċerebrosidażi, li jwassal għal żieda fil-livell ta’ sustanza glukosilċeramide f’partijiet differenti tal-ġisem, bħall-milsa, il-fwied u l-għadam.

gaucher disease is a rare inherited disorder, in which people do not have enough of an enzyme called acid beta-glucosidase, which normally breaks down a fatty waste product called glucosylceramide.
il-marda gaucher hija disturb rari li tintiret, li fiha persuna ma jkollhiex biżżejjed mill-enzima msejħa acid beta- glucosidase, li normalment tkisser prodott ta’ xaħam żejjed imsejjaħ glucosylceramide.

imiglucerase (recombinant macrophage targeted acid ß-glucosidase) replaces the deficient enzyme activity, hydrolysing glucosylceramide, thus correcting initial pathophysiology and preventing secondary pathology.
imiglucerase (makrofagu rikombinanti targeted acid ß-glucosidase) jissostitwixxi l-attività tal- enzima defiċjenti, jagħmel hydrolysing ta’ glucosylceramide, u għaldaqstant jikkoreġi l- patofiżjoloġija inizjali u jimpedixxi patoloġija sekondarja.

in individuals with gaucher disease, glucosylceramide degradation is insufficient, leading to accumulation of large quantities of this substrate within the lysosomes of macrophages (termed ‘gaucher cells’), leading to widespread secondary pathology.
f’individwi bil-marda ta’ gaucher, id-degradazzjoni ta’ glucosylceramide hi insuffiċjenti, u twassal għall-akkumulazzjoni ta’ kwantitajiet kbar ta’ dan is-substrat fil-lisosomi ta’ makrofaġi (imsejħa ‘ċelluli ta’ gaucher’), li jwasslu għal patoloġija sekondarja mifruxa.