Glycosphingolipid synthesis inhibition limits osteoclast activation and myeloma bone disease
File(s)JCI59987.pdf (5.61 MB)
Published version
Author(s)
Type
Journal Article
Abstract
Glycosphingolipids (GSLs) are essential constituents of cell membranes and lipid rafts and can modulate signal transduction events. The contribution of GSLs in osteoclast (OC) activation and osteolytic bone diseases in malignancies such as the plasma cell dyscrasia multiple myeloma (MM) is not known. Here, we tested the hypothesis that pathological activation of OCs in MM requires de novo GSL synthesis and is further enhanced by myeloma cell–derived GSLs. Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1. GM3 was the prevailing GSL produced by patient-derived myeloma cells and MM cell lines, and exogenous addition of GM3 synergistically enhanced the ability of the pro-osteoclastogenic factors RANKL and insulin-like growth factor 1 (IGF-1) to induce osteoclastogenesis in precursors. In WT mice, administration of GM3 increased OC numbers and activity, an effect that was reversed by treatment with NB-DNJ. In a murine MM model, treatment with NB-DNJ markedly improved osteolytic bone disease symptoms. Together, these data demonstrate that both tumor-derived and de novo synthesized GSLs influence osteoclastogenesis and suggest that NB-DNJ may reduce pathological OC activation and bone destruction associated with MM.
Date Issued
2015-04-27
Date Acceptance
2015-03-19
Citation
Journal of Clinical Investigation, 2015, 125 (6), pp.2279-2292
ISSN
1558-8238
Publisher
American Society for Clinical Investigation
Start Page
2279
End Page
2292
Journal / Book Title
Journal of Clinical Investigation
Volume
125
Issue
6
Copyright Statement
Copyright © 2015, The American Society for Clinical Investigation.
Sponsor
Biotechnology and Biological Sciences Research Council (BBSRC)
Biotechnology and Biological Sciences Research Council (BBSRC)
Grant Number
BB/F008309/1
BB/K016164/1
Subjects
Science & Technology
Life Sciences & Biomedicine
Medicine, Research & Experimental
Research & Experimental Medicine
MACROPHAGE INFLAMMATORY PROTEIN-1-ALPHA
CELL LINE HL-60
KAPPA-B LIGAND
MULTIPLE-MYELOMA
GAUCHER-DISEASE
RECEPTOR ACTIVATOR
N-BUTYLDEOXYNOJIRIMYCIN
SIGNAL-TRANSDUCTION
ALPHA MIP-1-ALPHA
PROTEIN 1-ALPHA
Publication Status
Published