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Butler, M.H.* ; Hayashi, A.* ; Ohkoshi, N.* ; Villmann, C.* ; Becker, C.M.* ; Feng, G.* ; de Camilli, P.* ; Solimena, M.*

Autoimmunity to gephyrin in Stiff-Man syndrome.

Neuron 26, 307-312 (2000)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
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
ISSN (print) / ISBN 0896-6273
e-ISSN 1097-4199
Journal Neuron
Quellenangaben Volume: 26, Issue: 2, Pages: 307-312 Article Number: , Supplement: ,
Publisher Elsevier
Publishing Place Cambridge, Mass.
Reviewing status Peer reviewed
Institute(s) Institute of Pancreatic Islet Research (IPI)