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Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases
Guimaraes Da Lomba Ferraz, M.J.; Marques, A.R.; Appelman, M.D.; Verhoek, M.; Strijland, A.; Mirzaian, M.; ... ; Aerts, J.M.F.G. 2016
Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases
Article / Letter to editor
metadata only
Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity of glycosphingolipid catabolizing glycosidases. We investigated the molecular basis of the... Show moreGlycosphingoid 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. Show less
Synthesis and Evaluation of Hybrid Structures Composed of Two Glucosylceramide Synthase Inhibitors
Berg, R.J.B.H.N. van der; Rijssel, E.R. van; Ferraz, M.J.; Houben, J.; Strijland, A.; Donker-Koopman, W.E.; ... ; Aerts, J.M.F.G. 2015
Synthesis and Evaluation of Hybrid Structures Composed of Two Glucosylceramide Synthase Inhibitors
Article / Letter to editor
metadata only
Ortho-Carborane-modified N-substituted Deoxynojirimycins
Hoogendoorn, S.; Mock, E.D.; Strijland, A.; Donker-Koopman, W.E.; Elst, H. van den; Berg, R.J.B.H.N. van den; ... ; Overkleeft, H.S. 2015
Ortho-Carborane-modified N-substituted Deoxynojirimycins
Article / Letter to editor
open access
2015-12-10T00:00:00Z
Action myoclonus-renal failure syndrome: diagnostic applications of activity-based probes and lipid analysis
Gaspar, P.; Kallemeijn, W.W.; Strijland, A.; Scheij, S.; Eijk, M. van; Aten, J.; ... ; Aerts, J.M.F.G. 2014
Action myoclonus-renal failure syndrome: diagnostic applications of activity-based probes and lipid analysis
Article / Letter to editor
open access
Elevated globotriaosylsphingosine is a hallmark of Fabry disease
Aerts, J.M.F.G.; Groener, J.E.; Kuiper, S.; Donker-Koopman, W.E.; Strijland, A.; Ottenhoff, R.; ... ; Poorthuis, B.J. 2008
Elevated globotriaosylsphingosine is a hallmark of Fabry disease
Article / Letter to editor
open access
Identification of the non-lysosomal glucosylceramidase as beta-glucosidase 2
Boot, R.G.; Verhoek, M.; Donker-Koopman, W.; Strijland, A.; Marle, J. van; Overkleeft, H.S.; ... ; Aerts, J.M.F.G. 2007
Identification of the non-lysosomal glucosylceramidase as beta-glucosidase 2
Article / Letter to editor
open access
Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase
Overkleeft, H.S.; Renkema, G.H.; Neele, J.; Vianello, P.; Hung, I.O.; Strijland, A.; ... ; Aerts, J.M.F.G. 1998
Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase
Article / Letter to editor
open access
The human chitotriosidase gene - Nature of inherited enzyme deficiency
Boot, R.G.; Renkema, G.H.; Verhoek, M.; Strijland, A.; Bliek, J.; Meulemeester, T.M.A.M.O. de; ... ; Aerts, J.M.F.G. 1998
The human chitotriosidase gene - Nature of inherited enzyme deficiency
Article / Letter to editor
open access
The human chitotriosidase gene - Nature of inherited enzyme deficiency
Boot, R.G.; Renkema, G.H.; Verhoek, M.; Strijland, A.; Bliek, J.; Meulemeester, T.M.A.M.O. de; ... ; Aerts, J.M.F.G. 1998
The human chitotriosidase gene - Nature of inherited enzyme deficiency
Article / Letter to editor
open access
Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase
Overkleeft, H.S.; Renkema, G.H.; Neele, J.; Vianello, P.; Hung, I.O.; Strijland, A.; ... ; Aerts, J.M.F.G. 1998
Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase
Article / Letter to editor
open access
Cloning of a cDNA Encoding Chitotriosidase, a Human Chitinase Produced by Macrophages
Boot, R.G.; Renkema, G.H.; Strijland, A.; Zonneveld, A.J. van; Aerts, J.M.F.G. 1995
Cloning of a cDNA Encoding Chitotriosidase, a Human Chitinase Produced by Macrophages
Article / Letter to editor
open access
Cloning of a cDNA Encoding Chitotriosidase, a Human Chitinase Produced by Macrophages
Boot, R.G.; Renkema, G.H.; Strijland, A.; Zonneveld, A.J. van; Aerts, J.M.F.G. 1995
Cloning of a cDNA Encoding Chitotriosidase, a Human Chitinase Produced by Macrophages
Article / Letter to editor
open access