Stiff-man syndrome: identification of 17β-hydroxysteroid dehydrogenase type 4 as a novel 80-kDa antineuronal antigen

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 wit...

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Hauptverfasser: Dinkel, Klaus (VerfasserIn) , Rickert, Mathias (VerfasserIn) , Möller, Gabriele (VerfasserIn) , Adamski, Jerzy (VerfasserIn) , Meinck, Hans-Michael (VerfasserIn) , Richter, Wiltrud (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 6 September 2002
In: Journal of neuroimmunology
Year: 2002, Jahrgang: 130, Heft: 1, Pages: 184-193
ISSN:1872-8421
DOI:10.1016/S0165-5728(02)00218-7
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/S0165-5728(02)00218-7
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S0165572802002187
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Verfasserangaben:Klaus Dinkel, Mathias Rickert, Gabriele Möller, Jerzy Adamski, Hans-Michael Meinck, Wiltrud Richter

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520 |a 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 17β-hydroxysteroid dehydrogenase type 4 and may represent a possible novel target of autoimmunity in SMS. 
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