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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: Accessed: January 23, 2018 Copyright © 2018 McGraw-Hill Education. All rights reserved
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