Abstract
The two possibilities to explain the pathogenic basis of stiff-person syndrome (SPS) are intrathecal sensitization of GAD65-reactive CD4+T cells and synthesis of GAD65-specific autoantibodies within the CNS [Rakocevic et al., Arch. Neurol. 61: 902-904, 2004]; and peripheral antigen sensitization followed by CNS antigen recognition by autoantibodies that cross the blood-brain barrier. Antigen-specific CD4+ T cells are essential for the generation of high-affinity autoantibodies [Lanzavecchia, Nature 314: 537-539, 1985], but there is no evidence of cellular infiltration in the CNS of SPS patients [Warich-Kirches et al., Clin. Neuropathol. 16: 214-219, 1997; Ishizawa et al., Acta Neuropathol.(Berl) 97: 63-70, 1999]. This review discusses the possible role of autoantibodies and autoreactive T cells specific to neuronal antigens in SPS pathogenesis.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 79-92 |
| Number of pages | 14 |
| Journal | International Reviews of Immunology |
| Volume | 27 |
| Issue number | 1-2 |
| DOIs | |
| State | Published - Jan 1 2008 |
| Externally published | Yes |
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Keywords
- Autoimmunity
- Gamma aminobutyric acid (GABA)
- Glutamic acid decarboxylase autoantibodies
- Stiff-person syndrome
- T cells
ASJC Scopus subject areas
- Immunology
Cite this
Immunobiology of stiff-person syndrome. / Raju, Raghavan Pillai; Hampe, Christiane S.
In: International Reviews of Immunology, Vol. 27, No. 1-2, 01.01.2008, p. 79-92.Research output: Contribution to journal › Review article
}
TY - JOUR
T1 - Immunobiology of stiff-person syndrome
AU - Raju, Raghavan Pillai
AU - Hampe, Christiane S.
PY - 2008/1/1
Y1 - 2008/1/1
N2 - The two possibilities to explain the pathogenic basis of stiff-person syndrome (SPS) are intrathecal sensitization of GAD65-reactive CD4+T cells and synthesis of GAD65-specific autoantibodies within the CNS [Rakocevic et al., Arch. Neurol. 61: 902-904, 2004]; and peripheral antigen sensitization followed by CNS antigen recognition by autoantibodies that cross the blood-brain barrier. Antigen-specific CD4+ T cells are essential for the generation of high-affinity autoantibodies [Lanzavecchia, Nature 314: 537-539, 1985], but there is no evidence of cellular infiltration in the CNS of SPS patients [Warich-Kirches et al., Clin. Neuropathol. 16: 214-219, 1997; Ishizawa et al., Acta Neuropathol.(Berl) 97: 63-70, 1999]. This review discusses the possible role of autoantibodies and autoreactive T cells specific to neuronal antigens in SPS pathogenesis.
AB - The two possibilities to explain the pathogenic basis of stiff-person syndrome (SPS) are intrathecal sensitization of GAD65-reactive CD4+T cells and synthesis of GAD65-specific autoantibodies within the CNS [Rakocevic et al., Arch. Neurol. 61: 902-904, 2004]; and peripheral antigen sensitization followed by CNS antigen recognition by autoantibodies that cross the blood-brain barrier. Antigen-specific CD4+ T cells are essential for the generation of high-affinity autoantibodies [Lanzavecchia, Nature 314: 537-539, 1985], but there is no evidence of cellular infiltration in the CNS of SPS patients [Warich-Kirches et al., Clin. Neuropathol. 16: 214-219, 1997; Ishizawa et al., Acta Neuropathol.(Berl) 97: 63-70, 1999]. This review discusses the possible role of autoantibodies and autoreactive T cells specific to neuronal antigens in SPS pathogenesis.
KW - Autoimmunity
KW - Gamma aminobutyric acid (GABA)
KW - Glutamic acid decarboxylase autoantibodies
KW - Stiff-person syndrome
KW - T cells
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U2 - 10.1080/08830180701883240
DO - 10.1080/08830180701883240
M3 - Review article
VL - 27
SP - 79
EP - 92
JO - International Reviews of Immunology
JF - International Reviews of Immunology
SN - 0883-0185
IS - 1-2
ER -