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

Authors: Dinkel, K  Rickert, M  Moller, G  Adamski, J  Meinck, HM  Richter, W 
Citation: Dinkel K, etal., J Neuroimmunol. 2002 Sep;130(1-2):184-93.
Pubmed: (View Article at PubMed) PMID:12225901

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

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RGD Object Information
RGD ID: 10411898
Created: 2015-11-10
Species: All species
Last Modified: 2015-11-10
Status: ACTIVE



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