Glucosylsphingosine

Glucosylsphingosine is the lyso-derivative of the common glycolipid glucocerebroside. This product is a fully synthetic standard containing only one isomer as opposed to glucosylsphingosine derived from natural sources. Glucosylceramide and glucosylsphingosine are important biological species that are the precursors for many complex glycosphingolipids. These lipids are involved in critical cellular functions such as cellular proliferation, differentiation, adhesion, signal transduction, cell-to-cell interactions, tumorigenesis, and metastasis. One of the most important roles of glucosylsphingosine is as a biomarker for the lysosomal storage disorder Gaucher disease. Gaucher disease is characterized by an accumulation of glucocerebroside due to a deficiency in the enzyme glucocerebrosidase and it has now been found that glucosylsphingosine also accumulates in this disease.1 This accumulation of glucopsychosine contributes to neuronal dysfunction and destruction in patients with neuronopathic Gaucher disease2 and it has been found to be a potent inhibitor of glucocerebrosidase. At least some instances of Gaucher disease also have a deficiency in the activity of glucosylsphingosine beta-glucosidase, the enzyme responsible for cleaving off the glucose of glucopsychosine and glucocerebroside. Like glucocerebroside and galactocerebroside, glucosylsphingosine can increase Ca2 + mobilization from intracellular stores although it uses a different mechanism.3 Conduritol B epoxide (CBE), an inhibitor of beta-glucosidase, and l-phenyl-2-decanoylamino-3-morpholino-l-propanol (PDMP), an inhibitor of glucosylceramide synthase, can be used to create a model of Gaucher disease and consequently an accumulation of glucosylsphingosine.4