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. 2012 Feb 2;2(1):134–164. doi: 10.3390/metabo2010134

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© 2012 by the authors; licensee MDPI, Basel, Switzerland.

This article is an open-access article distributed under the terms and conditions of the Creative Commons Attribution license (http://creativecommons.org/licenses/by/3.0/).

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Structures of two globo-series GSLs, Gb4Cer and the Forssman-GSL [26]. Only one of the various lipoforms is shown. Together with ganglioside GM2, Gb4Cer is a major storage substance in Sandhoff disease (β-hexosaminidase β-subunit deficiency). In humans, the Forssman-GSL, a blood group antigen, is present only in trace amounts together with globo-series and Lewis-type blood group glycolipids in the kidney [49]. In sheep, it is mainly responsible for haemolysis of erythrocytes in the presence of Gb4Cer antiserum. For example, in MDCK (Madin Darby canine kidney) cells, it plays a role in maintaining apical membrane transport, is absent in the plasma membrane of unpolarized MDCK cells and becomes the major GSL in their fully polarized state [50].