Figure 4.
Structure of globotriaosylceramide (Gb3) and analogues/isoforms. Gb3 with behenic acid (C22), as native Gb3 and methylated Gb3. Glu: glucose; Gal: galactose. The table shows that Gb3 isoforms/analogues, detected in plasma of Fabry patients, are statistically different from healthy controls [41]. Some of Gb3 analogues/isoforms have been found in urine of Fabry patients [68]. Gb3 Isoforms: Gb3 with varying length fatty acid chains joined by an amide linkage to an unmodified sphingosine moiety. The main sphingosine species are described as d18:1 = 18 carbon atoms with a single double bond. Gb3 Analogues: Gb3 has modified sphingosine moieties. The structure of Gb3 isoforms and analogues is expressed in the following way Gb3(dv:w)(Cx:y)Z, where d indicates the sphingosine group, v shows the number of carbons in the sphingosine moiety, w shows the number of double bonds in the sphingosine moiety, C indicates the fatty acid group, x shows the number of carbon atoms in the fatty acid moiety, y shows the number of carbon−carbon double bonds in the fatty acid moiety, and Z indicates other modifications related to the sphingosine structure (e.g., methylated (Me) or hydrated (H2O)). +: detected in plasma; ++: abundantly detected in plasma; +++: highly abundant in plasma; *: detected in urine; **: abundantly detected in urine; ***: highly abundant in urine; nd: not detected.