C18 Globotriaosylceramide-d3 (d18:1/18:0-d3)

C18 Globotriaosylceramide-d3 is intended for use as an internal standard for the quantification of C18 globotriaosylceramide by GC- or LC-MS. C18 globotriaosylceramide is an endogenous sphingolipid that accumulates in the brain, heart, kidney, liver, lung, and spleen in a mouse model of Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme alpha-galactosidase A. As this product is derived from a natural source, there may be variations in the sphingoid backbone. [Matreya, LLC. Catalog No. 1537]Formal Name: N-((2S,3R,E)-1-(((2R,3R,4R,5S,6R)-5-(((2S,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-(((2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)tetrahydro-2H-pyran-2-yl)oxy)tetrahydro-2H-pyran-2-yl)oxy)-3,4-dihydroxy-6-(hydroxymethyl)tetrahydro-2H-pyran-2-yl)oxy)-3-hydroxyoctadec-4-en-2-yl)octadecanamide-d3. Synonyms: N-omega-CD3-Octadecanoyl-ceramide Trihexoside, Galalpha1-4Galbeta1-4Glcbeta-Cer(d18:1/18:0) d3, Gb3(d18:1/18:0-d3), Globotriaosylceramide-d3 (d18:1/18:0-d3). Molecular Formula: C54H98D3NO18. Formula Weight: 1055.4. Purity: >99% deuterated forms (d1-d3). Formulation: (Request formulation change), A solid. Solubility: Chloroform:Methanol (2:1): soluble, DMSO: soluble. SMILES: O[C@H]1[C@H](O[C@]2([H])O[C@H](CO)[C@H](O[C@]3([H])[C@H](O)[C@@H](O)[C@@H](O)[C@@H](CO)O3)[C@H](O)[C@H]2O)[C@@H](CO)O[C@@H](OC[C@H](NC(CCCCCCCCCCCCCCCCC([2H])([2H])[2H])=O)[C@H](O)/C=C/CCCCCCCCCCCCC)[C@@H]1O. InChi Code: InChI=1S/C54H101NO18/c1-3-5-7-9-11-13-15-17-18-20-22-24-26-28-30-32-42(60)55-37(38(59)31-29-27-25-23-21-19-16-14-12-10-8-6-4-2)36-68-52-48(66)45(63)50(40(34-57)70-52)73-54-49(67)46(64)51(41(35-58)71-54)72-53-47(65)44(62)43(61)39(33-56)69-53/h29,31,37-41,43-54,56-59,61-67H,3-28,30,32-36H2,1-2H3,(H,55,60)/b31-29+/t37-,38+,39+,40+,41+,43-,44-,45+,46+,47+,48+,49+,50+,51-,52+,53+,54-/m0/s1/i1D3. InChi Key: KWGVNZWSEWAOMI-TUISFQHUSA-N.