Glucosylsphingosine Causes Hematological and Visceral Changes in Mice—Evidence for a Pathophysiological Role in Gaucher Disease

Glucosylsphingosine Causes Hematological and Visceral Changes in Mice—Evidence for a Pathophysiological Role in Gaucher Disease

Glucosylceramide and glucosylsphingosine are the two major storage products in Gaucher disease (GD), an inherited metabolic disorder caused by a deficiency of the lysosomal enzyme glucocerebrosidase. The build-up of glucosylceramide in the endoplasmic reticulum and prominent accumulation in cell lys...

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Bibliographic Details
Main Authors: Jan Lukas, Claudia Cozma, Fan Yang, Guido Kramp, Anja Meyer, Anna-Maria Neßlauer, Sabrina Eichler, Tobias Böttcher, Martin Witt, Anja U. Bräuer, Peter Kropp, Arndt Rolfs
Format: Article
Language:English
Published: MDPI AG 2017-10-01
Series:International Journal of Molecular Sciences
Subjects:
sphingolipids
glucocerebrosidase
biomarker
storage disease pathology
chemically-induced phenotype
Online Access:https://www.mdpi.com/1422-0067/18/10/2192

Internet

https://www.mdpi.com/1422-0067/18/10/2192

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