AUTOANTIBODIES TO GLUTAMATE-RECEPTOR GLUR3 IN RASMUSSENS ENCEPHALITIS

AUTOANTIBODIES TO GLUTAMATE-RECEPTOR GLUR3 IN RASMUSSENS ENCEPHALITIS
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DOI:
10.1126/science.8036512
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发表时间:
1994-07-29
期刊:
影响因子:
56.9
通讯作者:
MCNAMARA, JO
MCNAMARA, JO
中科院分区:
综合性期刊1区
文献类型:
--
作者:
ROGERS, SW;ANDREWS, PI;MCNAMARA, JO

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Rasmussen's encephalitis is a progressive childhood disease of unknown cause characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain. During efforts to raise antibodies to recombinant glutamate receptors (GluRs), behaviors typical of seizures and histopathologic features mimicking Rasmussen's encephalitis were found in two rabbits immunized with GluR3 protein. A correlation was found between the presence of Rasmussen's encephalitis and serum antibodies to GluR3 detected by protein immunoblot analysis and by immunoreactivity to transfected cells expressing GluR3. Repeated plasma exchanges in one seriously ill child transiently reduced serum titers of GluR3 antibodies, decreased seizure frequency, and improved neurologic function. Thus, GluR3 is an autoantigen in Rasmussen's encephalitis, and an autoimmune process may underlie this disease.