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Critical Reviews™ in Oncogenesis

Erscheint 4 Ausgaben pro Jahr

ISSN Druckformat: 0893-9675

ISSN Online: 2162-6448

SJR: 0.395 SNIP: 0.322 CiteScore™:: 2.5 H-Index: 54

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Gaucher's Disease and Cancer: A Sphingolipid Perspective

Volumen 18, Ausgabe 3, 2013, pp. 221-234
DOI: 10.1615/CritRevOncog.2013005814
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ABSTRAKT

Gaucher's disease is a sphingolipidosis characterized by a specific deficiency in an acidic glucocerebrosidase, which results in aberrant accumulation of glucosylceramide primarily within the lysosome. Gaucher's disease has been correlated with cases of myeloma, leukemia, glioblastoma, lung cancer, and hepatocellular carcinoma, although the reasons for the correlation are currently being debated. Some suggest that the effects of Gaucher's disease may be linked to cancer, while others implicate the therapies used to treat Gaucher's disease. This debate is not entirely surprising, as the speculations linking Gaucher's disease with cancer fail to address the roles of ceramide and glucosylceramide in cancer biology. In this review, we will discuss, in the context of cancer biology, ceramide metabolism to glucosylceramide, the roles of glucosylceramide in multidrug-resistance, and the role of ceramide as an anticancer lipid. This review should reveal that it is most practical to associate elevated glucosylceramide, which accompanies Gaucher's disease, with the progression of cancer. Furthermore, this review proposes that the therapies used to treat Gaucher's disease, which augment ceramide accumulation, are likely not linked to correlations with cancer.

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