摘要 |
Disclosed are compounds of formula I where the substituents are as defined herein. The compounds are predominantly quinuclidine derivatives for example quinuclidin-3-yl(2-(4’-fluoro-[1,1’-biphenyl]-3- yl)propan-2-yl)carbamate. The compounds of the invention are inhibitors of glucosylceramide synthase (GCS) and are intended for the treatment metabolic diseases, such as lysosomal storage diseases, either alone or in combination with enzyme replacement therapy, and for the treatment of cancer. |