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Open Access Green as soon as Postprint is submitted to ZB.
Autoimmunity to gephyrin in Stiff-Man syndrome.
Neuron 26, 307-312 (2000)
Stiff-Man syndrome (SMS) is a rare disease of the central nervous system (CNS) characterized by chronic rigidity, spasms, and autoimmunity directed against synaptic antigens, most often the GABA-synthesizing enzyme glutamic acid decarboxylase (GAD). In a subset of cases, SMS has an autoimmune paraneoplastic origin. We report here the identification of high-titer autoantibodies directed against gephyrin in a patient with clinical features of SMS and mediastinal cancer. Gephyrin is a cytosolic protein selectively concentrated at the postsynaptic membrane of inhibitory synapses, where it is associated with GABA(A) and glycine receptors. Our findings provide new evidence for a close link between autoimmunity directed against components of inhibitory synapses and neurological conditions characterized by chronic rigidity and spasms.
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Publication type
Article: Journal article
Document type
Scientific Article
Language
english
Publication Year
2000
HGF-reported in Year
0
ISSN (print) / ISBN
0896-6273
e-ISSN
1097-4199
Journal
Neuron
Quellenangaben
Volume: 26,
Issue: 2,
Pages: 307-312
Publisher
Cell Press
Publishing Place
Cambridge, Mass.
Reviewing status
Peer reviewed
Institute(s)
Institute of Pancreatic Islet Research (IPI)
PubMed ID
10839351
Erfassungsdatum
2000-12-31