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 A LS, although the pathogenetic significance of the antibodies is still unkno wn. We have already demonstrated that SGPG, a unique glycolipid, is present in both peripheral nerve and vascular endothelial cells. To investigate wh ether serum anti-SGPG antibodies would participate in activation and/or inj ury of endothelial cells in ALS, we examined serum anti-SGPG antibodies in association with serum soluble E- and P-selectins, which are markers of act ivated endothelial cells, in 25 patients with ALS and 14 age-matched patien ts with other neurological diseases (ONDs) using the microtiter-ELISA metho d. Seven out of 25 ALS patients had anti-SGPG antibodies. Levels of sE-sele ctin were significantly higher in patients with ALS (48.5 +/- 23.4 ng/ml) c ompared 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 level s. The mean sE-selectin levels were higher in patients with anti-SGPG antib odies (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.