[13C6]-Glucosylsphingosine ([13C6]-GlSph)

Glucosylsphingosine (GlcSph) is a key biomarker of Gaucher Disease (GD). Glucosylsphingosine is a cytotoxic compound. Accumulation of glucosylsphingosine in brain and other tissues occurs in patients with Gaucher disease, which is an inherited deficiency of lysosomal glucocerebrosidase, which converts glucosylsphingosine to glucose and sphingosine. Stable isotope labeled Glucosylsphingosine (GlcSph) is intended for use as an internal standard for the quantification of Glucosylsphingosine by LC or GC-mass spectrometry.