A globotriaosylceramide lacking the fatty acyl group; binds to Stx1 in the presence of cholesterol and phosphatidylcholine, but does not bind Stx2; reduces viability and PMA-induced aggregation of human neutrophils (50 and 1 μM, respectively); accumulates in various organs in a mouse model of Fabry disease; accumulates in urine, kidneys, and plasma of patients with Fabry disease; decreases in response to administration of 1-deoxygalactonojirimycin in a transgenic mouse model of Fabry disease; has been used as a biomarker for efficacy of ERT and other therapies in the treatment of Fabry disease,