Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases - Ferraz, Maria J.; Marques, André R. A.; Appelman, Monique D.; Verhoek, Marri; Strijland, Anneke; Mirzaian, Mina; Scheij, Saskia; Ouairy, Cécile M.; Lahav, Daniel; Wisse, Patrick; Overkleeft, Herman S.; Boot, Rolf G.; et al. (FEBS letters, 2016)
Keywords: acid ceramidase; Acid Ceramidase - genetics; Acid Ceramidase - metabolism; Acylation; alpha-Galactosidase - genetics; alpha-Galactosidase - metabolism; Animals; Disease Models, Animal; Fabry disease; Fabry Disease - genetics; Fabry Disease - metabolism; Female; Gaucher disease; Gaucher Disease - genetics; Gaucher Disease - metabolism; globotriaosylsphingosine; Glucosylceramidase - genetics; Glucosylceramidase - metabolism; glucosylsphingosine; glycosphingolipids; Glycosphingolipids - chemistry; Glycosphingolipids - metabolism; HEK293 Cells; Humans; Lysosomes - metabolism; Male; Mice; Mice, Knockout; Fabry disease; glucosylsphingosine; Gaucher disease; globotriaosylsphingosine; glycosphingolipids; acid ceramidase