Elevation of serum soluble E‐selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis

Abstract

Immunological abnormality is often found in amyotrophic lateral sclerosis (ALS). Antibodies to sulfoglucuronyl paragloboside (SGPG) were reported in ALS, although the pathogenetic significance of the antibodies is still unknown. We have already demonstrated that SGPG, a unique glycolipid, is present in both peripheral nerve and vascular endothelial cells. To investigate whether serum anti-SGPG antibodies would participate in activation and/or injury of endothelial cells in ALS, we examined serum anti-SGPG antibodies in association with serum soluble E- and P-selectins, which are markers of activated endothelial cells, in 25 patients with ALS and 14 age-matched patients with other neurological diseases (ONDs) using the microtiter-ELISA method. Seven out of 25 ALS patients had anti-SGPG antibodies. Levels of sE-selectin were significantly higher in patients with ALS (48.5 +/- 23.4 ng/ml) compared with ONDs (24.0 +/- 11.8 ng/ml) (P < 0.005). Four out of seven ALS patients with anti-SGPG antibodies had concomitantly high sE-selectin levels. The mean sE-selectin levels were higher in patients with anti-SGPG antibodies (61.9 +/- 25.2 ng/ml) than in those without anti-SGPG antibodies (43.3 +/- 21.1 ng/ml). Anti-SGPG antibodies may take part in the activation and/or injury of endothelial cells. The increased expression of E-selectin may be related to an immunological process in some ALS patients.

The role of soluble intercellular adhesion molecules in neurodegenerative disorders

“…However, occasionally patients with motor neuron disorders or clinically similar neuropathies have responded to immunosuppressive therapy [60][61][62]. Our study has not confirmed the results of a recent study [26] dealing with the role of E-selectin in ALS. The nonincreased levels of s-ICAM and s-E-selectin in our patients do not implicate endothelial activation and probably cytokine's production in the pathogenesis of ALS.…”

“…Serum and cerebrospinal fluid (CSF) immunological findings were analyzed [25], and elevated CSF IgG index, higher incidence of immune complexes, complement involvement and CSF oligoclonal bands were reported. Recently, an elevation of serum soluble E-selectin associated with antibodies to sulfoglucuronyl paragloboside (SGPG) was reported in patients with ALS [26]. These results are an evidence of endothelial cell activation in some ALS patients.…”

Anti-Sulfoglucuronosyl Paragloboside Antibody

“…Sulfoglucuronosyl paragloboside (SGPG) is one of the glycolipid target antigens in various peripheral neuropathies ( Leger et al., 1996 ). Anti-SGPG antibody has been reported by us as well as other groups to be present in the serum samples of some ALS patients ( Maeda et al., 1991 ; Ben Younes-Chennoufi et al., 1995 ; Ikeda et al., 2000 ). However, its involvement in the neuropathogenesis of ALS has not been studied in a large number of patients with well-documented clinical and demographic records, which precludes its clinical significance.…”

Cerebrospinal fluid and serum antibodies against neurofilaments in patients with amyotrophic lateral sclerosis

“…In our studies with patients having multiple sclerosis, we demonstrated elevated levels of intrathecal anti‐NFM [13]. The immune response is not only directed against cytoskeletal structures, but also other neuron components such as gangliosides [3,16,17].…”