4LSC2610 [13C6]-Glucosylsphingosine CAS# 299172-48-8

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.

If the number, nature and/or location of stable isotope label atoms (D, ¹⁵N, ¹³C) in this molecule don’t fit with your application, don’t hesitate to contact us to discuss your specific needs.

Keywords:

Clinical chemistry, diagnostic tools & biomarkers, stable isotope dilution analysis, internal standard, stable isotope labeled standard (SIL), therapeutic drug monitoring (TDM), quantitative bioanalytical method validation, clinical diagnostic, LC/MS method validation.

• Molecular Formula: C₁₈¹³C₆H₄₇NO₇
• Molecular Weight: 467.59 g/mol
• Specification:
  • Minimum purity: 95.0 %
  • Minimum Isotopic Enrichment: ¹³C ≥99 %
• HS Code: 284590
• Packing Size: 1mg, 5mg, 25mg, bulk
• Solubility: ethanol, methanol, chloroform/methanol (2:1)
• Physical Appearance: solid
• Storage: -20°C

Glucosylsphingosine is a highly sensitive and specific biomarker for primary diagnostic and follow-up monitoring in Gaucher disease in a non-Jewish, Caucasian cohort of Gaucher disease patients. Rolfs et al., 2013, PLoS One., 8(11):e79732. doi: 10.1371/journal.po10.1371

Glucosylsphingosine is a key biomarker of Gaucher disease. Murugesan et al., 2016, Am. J. Hematol., 91(11):1082-1089. doi: 10.1002/ajh.24491

Glucosylceramide and Glucosylsphingosine Quantitation by Liquid Chromatography-Tandem Mass Spectrometry to Enable In Vivo Preclinical Studies of Neuronopathic Gaucher Disease. Hamler et al., 2017, Anal Chem., 15; 89(16):8288-8295. doi: 10.1021/acs.analchem.7b01442