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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Main Authors: Dinkel, Klaus (Author) , Rickert, Mathias (Author) , Möller, Gabriele (Author) , Adamski, Jerzy (Author) , Meinck, Hans-Michael (Author) , Richter, Wiltrud (Author)
Format: Article (Journal)
Language:English
Published: 6 September 2002
In: Journal of neuroimmunology
Year: 2002, Volume: 130, Issue: 1, Pages: 184-193
ISSN:1872-8421
DOI:10.1016/S0165-5728(02)00218-7
Online Access: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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Author Notes:Klaus Dinkel, Mathias Rickert, Gabriele Möller, Jerzy Adamski, Hans-Michael Meinck, Wiltrud Richter
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Summary: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.
Item Description:Gesehen am 01.03.2021
Physical Description:Online Resource
ISSN:1872-8421
DOI:10.1016/S0165-5728(02)00218-7