N-Glycinated glucosylsphingosine

N-Glycinated glucosylsphingosine is an analogue of the important biomolecule glucosylsphingosine. It is ideal for use as an internal standard in the extraction and mass spectrometry analysis of glucosylsphingosine from natural samples.(1) The free amine group gives this product very similar physical characteristics to the natural glycolipid while the glycine adds an additional 57 units to the molecule making it easy to detect by MS. 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.(2) This accumulation of glucopsychosine contributes to neuronal dysfunction and destruction in patients with neuronopathic Gaucher disease(3) 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.(4)