Complete chemical structures of the neutral glycosphingolipids that accumulate in Fabry disease. A, Globotriaosylceramide, the major accumulated substrate. B, Galabiosylceramide. C and D, The blood group B and B1 antigenic glycosphingolipids, respectively, which accumulate in blood group B and AB patients. The arrows indicate the α-galactosyl bonds, which are normally cleaved by α-galactosidase A. Source: α-Galactosidase A Deficiency: Fabry Disease, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: https://ommbid.mhmedical.com/DownloadImage.aspx?image=/data/books/971/ch150fg10.png&sec=62644922&BookID=971&ChapterSecID=62644837&imagename=&gboscontainerID=0 Accessed: January 23, 2018 Copyright © 2018 McGraw-Hill Education. All rights reserved