Autoimmunity to munc-18 in Rasmussen's encephalitis.

Published

Journal Article

Rasmussen's encephalitis (RE) is a rare disease of the central nervous system characterized by severe epileptic seizures, progressive degeneration of a single cerebral hemisphere, and autoimmunity directed against glutamate receptor subunit, GluR3. We report here the identification of high-titer autoantibodies directed against munc-18 in the serum of a single patient with RE previously shown to have anti-GluR3 antibodies. Munc-18 is an intracellular protein residing in presynaptic terminals, which is required for secretion of neurotransmitters. These findings are consistent with the possibility of intermolecular epitope spreading between GluR3, a postsynaptic cell surface protein, and munc-18, a presynaptic intracellular protein. Immune attack on these two proteins, which participate at distinct steps of synaptic transmission, could act in an additive or synergistic manner to impair synaptic function and lead to seizures and neuronal death.

Full Text

Duke Authors

Cited Authors

  • Yang, R; Puranam, RS; Butler, LS; Qian, WH; He, XP; Moyer, MB; Blackburn, K; Andrews, PI; McNamara, JO

Published Date

  • November 2000

Published In

Volume / Issue

  • 28 / 2

Start / End Page

  • 375 - 383

PubMed ID

  • 11144349

Pubmed Central ID

  • 11144349

International Standard Serial Number (ISSN)

  • 0896-6273

Digital Object Identifier (DOI)

  • 10.1016/s0896-6273(00)00118-5

Language

  • eng

Conference Location

  • United States