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Autoimmunity to munc-18 in Rasmussen's encephalitis
R Yang1, R S Puranam, L S Butler
1Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710, USA.
Neuron
|January 6, 2001
Summary
Rasmussen's encephalitis (RE) patients may have autoantibodies against both GluR3 and munc-18 proteins. This dual attack on synaptic proteins could worsen seizures and neuronal death in this rare neurological disease.
Area of Science:
- Neuroimmunology
- Neurology
- Molecular Neuroscience
Background:
- Rasmussen's encephalitis (RE) is a rare autoimmune CNS disease.
- It involves seizures, hemisphere degeneration, and autoantibodies against the postsynaptic glutamate receptor subunit GluR3.
Observation:
- A single RE patient exhibited high-titer autoantibodies against munc-18.
- This patient also had previously identified anti-GluR3 antibodies.
Findings:
- Munc-18 is an intracellular presynaptic protein crucial for neurotransmitter secretion.
- The presence of autoantibodies against both GluR3 (postsynaptic) and munc-18 (presynaptic) suggests intermolecular epitope spreading.
- This dual immune attack targets distinct steps in synaptic transmission.
Implications:
- Co-targeting of GluR3 and munc-18 may synergistically impair synaptic function.
- This could contribute to the severe seizures and neuronal death observed in Rasmussen's encephalitis.
- Further research into these autoantibodies may reveal new diagnostic or therapeutic targets for RE.
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