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detta enzym bryter normalt ner en fettsubstans som kallas globotriaosylceramid (gb3).
this enzyme normally breaks down a fatty substance called globotriaosylceramide (gb3).
Last Update: 2012-04-11
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om du lider av fabrys sjukdom avlägsnas inte ett fettämne, som heter globotriaosylceramid (gl- 3), från cellerna i din kropp ansamlas i stället i dina organs blodkärlsväggar.
if you suffer from fabry disease a fat substance, called globotriaosylceramide (gl-3), is not removed from the cells of your body and starts to accumulate in the walls of the blood vessels of your organs.
Last Update: 2012-04-11
Usage Frequency: 2
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fabrys sjukdom är en rubbning av glykosfingolipidlagringen som orsakas av bristande aktivitet hos lysosomenzymet α- galaktosidas a, vilket leder till ackumulation av globotriaosylceramid (aven kallad gb3 eller cth), glykosfingolipidsubstratet för detta enzym.
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 (also referred to as gb3 or cth), the glycosphingolipid substrate for this enzyme.
Last Update: 2012-04-11
Usage Frequency: 2
Quality: