Glycosphingolipid depletion in PC12 cells using iminosugars protects neuronal membranes from anti-ganglioside antibody mediated injury
KH Townson, AO Speak, KN Greenshields… - Journal of …, 2008 - Elsevier
Autoimmune neuropathies are frequently associated with pathogenic anti-ganglioside
antibodies targeting ganglioside-rich neuronal and glial membranes. The extent of injury is
determined by the concentration of membrane ganglioside and thus reduction might be
expected to attenuate disease. In this study, we suppressed ganglioside biosynthesis in
PC12 cells with the glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin and
observed reduced plasma membrane antibody binding and a major neuroprotective effect in …
antibodies targeting ganglioside-rich neuronal and glial membranes. The extent of injury is
determined by the concentration of membrane ganglioside and thus reduction might be
expected to attenuate disease. In this study, we suppressed ganglioside biosynthesis in
PC12 cells with the glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin and
observed reduced plasma membrane antibody binding and a major neuroprotective effect in …