Reduced soluble RAGE is associated with disease severity of axonal Guillain-Barré syndrome.

Da-Qi Zhang,Ting Li,Li Yang,Guo-Qiang Chang,Jian-Ping Zhou,Ning Zhao,Rong Wang,Hui Zhai,Li-Na Yang

doi:10.1038/srep21890

Abstract

Soluble receptor for advanced glycation end products (sRAGE) is an anti-inflammatory factor that mitigates the proinflammatory effects of high mobility group box 1 (HMGB1). The aim of this study was to investigate whether Guillain-Barré syndrome (GBS)-related inflammation are mediated by sRAGE and HMGB1. We measured serum sRAGE, HMGB1, IL-6, and TNF-α levels in 86 patients with GBS and analysed associations between sRAGE or HMGB1 and clinical variables in these subjects. In addition, we determined cerebrospinal fluid sRAGE and HMGB1 levels in a cross-sectional study of 50 patients with GBS who had matched serum samples. We found serum sRAGE levels in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS, but not other subtypes, were significantly lower than those in healthy controls, and were significantly correlated with GBS disability score and Erasmus GBS outcome score, while serum HMGB1, IL-6, and TNF-α levels in all subtypes of GBS were significantly higher than those in healthy controls. Moreover, increased sRAGE levels and decreased HMGB1 levels after treatment were observed. Our results showed that serum sRAGE may be a useful biomarker for inflammation in the AMAN GBS subtype, while HMGB1 may be related to the inflammatory process across all types of GBS.

Highlights

Guillain-Barré syndrome (GBS) is an acute, post-infectious, immune-mediated spectrum disorder that affects the peripheral nervous system
When comparing the GBS disability score and Erasmus GBS outcome score (EGOS) among the acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and unclassified patients with GBS, we found that the GBS disability scores and EGOS in AMAN were significantly higher than those in AIDP and the unclassified subtype (p < 0.001, respectively, Table 1)
Our results showed that serum levels of soluble receptor for advanced glycation end products (RAGE) (sRAGE) were significantly decreased only in the AMAN subtype of GBS

Summary

Introduction

Guillain-Barré syndrome (GBS) is an acute, post-infectious, immune-mediated spectrum disorder that affects the peripheral nervous system. Human RAGE is expressed as several different isoforms: a full-length membrane-bound RAGE(mRAGE) which consists of an extracellular, transmembrane, and cytosolic domain; a N-truncated RAGE and a C-truncated RAGE without a transmembrane and cytosolic domain The latter of these, which can be released extracellularly, is called soluble RAGE (sRAGE)[9]. To investigate whether sRAGE or HMGB1 is involved in GBS-related inflammation in a general or subtype-specific manner, we determined their serum and CSF levels in subtypes of GBS.

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