Lyso GB3-d7

• CAT Number: I043543
• CAS Number: 2315262-44-1
• Molecular Formula: C36H60D7NO17
• Molecular Weight: 792.96
• Purity: ≥95%
• Synonyms: (2R,3R,4S,5R,6R)-2-[(2R,3R,4R,5R,6S)-6-[(2R,3S,4R,5R,6R)-6-[(E,2S,3R)-2-amino-16,16,17,17,18,18,18-heptadeuterio-3-hydroxyoctadec-4-enoxy]-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy-4,5-dihydroxy-2-(hydroxymethyl)oxan-3-yl]oxy-6-(hydroxymethyl)oxane-3,4,5-triol
• InChI: InChI=1S/C36H67NO17/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-21(41)20(37)19-49-34-30(47)27(44)32(23(17-39)51-34)54-36-31(48)28(45)33(24(18-40)52-36)53-35-29(46)26(43)25(42)22(16-38)50-35/h14-15,20-36,38-48H,2-13,16-19,37H2,1H3/b15-14+/t20-,21+,22+,23+,24+,25-,26-,27+,28+,29+,30+,31+,32+,33-,34+,35+,36-/m0/s1/i1D3,2D2,3D2
• InChIKey: GRGNVOCPFLXGDQ-IZKFDWHNSA-N
• SMILES: [2H]C([2H])([2H])C([2H])([2H])C([2H])([2H])CCCCCCCCCC/C=C/[C@H]([C@H](CO[C@H]1[C@@H]([C@H]([C@@H]([C@H](O1)CO)O[C@H]2[C@@H]([C@H]([C@H]([C@H](O2)CO)O[C@@H]3[C@@H]([C@H]([C@H]([C@H](O3)CO)O)O)O)O)O)O)O)N)O

Lyso GB3-d7（Cat No.:I043543）is a deuterated analog of lyso-globotriaosylceramide (Lyso GB3), a glycosphingolipid that accumulates in tissues in patients with Fabry disease. The deuterium-labeled version (Lyso GB3-d7) is used in metabolic studies, particularly to track and analyze the metabolic pathways and turnover of Lyso GB3 in vivo. It serves as a tool in research aimed at understanding the pathophysiology of Fabry disease and developing potential therapies. Lyso GB3-d7 provides valuable insights into the disease’s progression and can aid in evaluating new treatment strategies for this rare lysosomal storage disorder.