Globotriaosylceramid​e Sphingosine-d2,13C tert-Butyldimethylsilyl

• CAT Number: R061331
• CAS Number: NA
• Molecular Formula: C₄₁¹³CH₇₉D₂NO₁₇Si
• Molecular Weight: 903.18
• Purity: ≥95%
• Synonyms: (2S,​3R,​4E)​-​2-​Amino-​3-​((tert-butyldimethylsilyl)oxy)-​4-​octadecenyl-d2,13C O-​α-​D-​Galactopyranosyl-​(1→4)​-​O-​β-​D-​galactopyranosyl-​(1→4)​-β-​D-​glucopyranoside; lyso-​Gb3-d2,13C tert-Butyldimethylsilyl; (2R,3R,4S,5R,6R)-2-(((2R,3R,4R,5R,
• Storage: -20°C

Globotriaosylceramide Sphingosine-d2,13C tert-Butyldimethylsilyl is a highly specialized isotopically labeled compound used in the study of lysosomal storage disorders, particularly Fabry disease. This compound features deuterium and carbon-13 isotopes, making it an ideal internal standard for mass spectrometry-based analyses. The tert-butyldimethylsilyl (TBDMS) protection enhances the stability and volatility of the molecule, facilitating its use in GC-MS and LC-MS applications. Globotriaosylceramide Sphingosine-d2,13C TBDMS is essential for precise quantification in complex biological matrices, aiding in the investigation of glycosphingolipid metabolism, enzyme assays, and the development of therapeutic strategies.