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

被引：17

作者：

Dinkel, K

Rickert, M

Möller, G

Adamski, J

Meinck, HM

Richter, W

机构：

[1] Heidelberg Univ, Dept Orthoped Surg, D-69119 Heidelberg, Germany

[2] GSF, Natl Res Ctr Environm & Hlth, Inst Expt Genet, D-85758 Neuherberg, Germany

[3] Heidelberg Univ, Dept Neurol, D-69120 Heidelberg, Germany

来源：

JOURNAL OF NEUROIMMUNOLOGY | 2002年 / 130卷 / 1-2期

关键词：

human monoclonal antibodies; autoimmune disease; mass spectrometry;

D O I：

10.1016/S0165-5728(02)00218-7

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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. (C) 2002 Elsevier Science B.V. All rights reserved.

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页码：184 / 193

页数：10

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