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

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.