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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 complement-mediated lysis assays. These data demonstrate that iminosugar inhibitors, currently used to treat type 1 Gaucher disease, are also of potential value for depleting antigen and thereby suppressing tissue injury in anti-ganglioside antibody-associated neuropathy.

Original publication

DOI

10.1016/j.jneuroim.2008.06.033

Type

Journal article

Journal

J Neuroimmunol

Publication Date

15/10/2008

Volume

203

Pages

33 - 38

Keywords

1-Deoxynojirimycin, Animals, Antibodies, Cell Membrane, Complement System Proteins, Enzyme Inhibitors, Gangliosides, Glycosphingolipids, Imino Sugars, Neuritis, Autoimmune, Experimental, Neurons, Neuroprotective Agents, PC12 Cells, Rats