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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Detalles Bibliográficos
Autores principales: Lukas, Jan, Cozma, Claudia, Yang, Fan, Kramp, Guido, Meyer, Anja, Neßlauer, Anna-Maria, Eichler, Sabrina, Böttcher, Tobias, Witt, Martin, Bräuer, Anja U., Kropp, Peter, Rolfs, Arndt
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666873/
https://www.ncbi.nlm.nih.gov/pubmed/29053611
http://dx.doi.org/10.3390/ijms18102192

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666873/
https://www.ncbi.nlm.nih.gov/pubmed/29053611
http://dx.doi.org/10.3390/ijms18102192

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