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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; Aerts, Johannes M.
Afiliación
  • Ferraz MJ; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
  • Marques AR; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
  • Appelman MD; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
  • Verhoek M; Department of Medical Biochemistry, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Strijland A; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
  • Mirzaian M; Department of Medical Biochemistry, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Scheij S; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
  • Ouairy CM; Department of Bio-organic Synthesis, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Lahav D; Department of Bio-organic Synthesis, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Wisse P; Department of Bio-organic Synthesis, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Overkleeft HS; Department of Bio-organic Synthesis, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Boot RG; Department of Medical Biochemistry, Leiden Institute of Chemistry, Leiden University, The Netherlands.
  • Aerts JM; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
FEBS Lett ; 590(6): 716-25, 2016 Mar.
Article en En | MEDLINE | ID: mdl-26898341
Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity of glycosphingolipid catabolizing glycosidases. We investigated the molecular basis of the formation of glucosylsphingosine and globotriaosylsphingosine during deficiency of glucocerebrosidase (Gaucher disease) and α-galactosidase A (Fabry disease). Independent genetic and pharmacological evidence is presented pointing to an active role of acid ceramidase in both processes through deacylation of lysosomal glycosphingolipids. The potential pathophysiological relevance of elevated glycosphingoid bases generated through this alternative metabolism in patients suffering from lysosomal glycosidase defects is discussed.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glicoesfingolípidos / Enfermedad de Fabry / Ceramidasa Ácida / Enfermedad de Gaucher Límite: Animals / Female / Humans / Male Idioma: En Revista: FEBS Lett Año: 2016 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glicoesfingolípidos / Enfermedad de Fabry / Ceramidasa Ácida / Enfermedad de Gaucher Límite: Animals / Female / Humans / Male Idioma: En Revista: FEBS Lett Año: 2016 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido