PuSH - Publication Server of Helmholtz Zentrum München: Stiff-man syndrome : Identification of 17ß-hydroxysteroid dehydrogenase type 4 as a novel 80-kDa antineuronal antigen.

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Dinkel, K.* ; Rickert, M.* ; Möller, G. ; Adamski, J. ; Meinck, H.-M.* ; Richter, W.*

Stiff-man syndrome : Identification of 17ß-hydroxysteroid dehydrogenase type 4 as a novel 80-kDa antineuronal antigen.

J. Neuroimmunol. 130, 184-193 (2002)

Open Access Green as soon as Postprint is submitted to ZB.

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Stiff-man syndrome (SMS) is a rare autoimmune disorder of the central nervous system associated with autoantibodies to glutamate decarboxylase (GAD). We isolated five brain-reactive human monoclonal antibodies, with reactivity distinct from GAD, from peripheral blood of a patient newly diagnosed with SMS. Two antibodies reacted with both Purkinje cells and ependymal cells, and precipitated an 80-kDa protein from rat neuronal primary cultures, which was also recognized by 12% (3/25) of SMS sera and 13% (2/15) of SMS cerebrospinal fluid (CSF) samples. The corresponding antigen was identified as 17beta-hydroxysteroid dehydrogenase type 4 and may represent a possible novel target of autoimmunity in SMS.

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