Globotriaosylceramide

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The structural formula of the Globotriaosylceramide

Globotriaosylceramide , also known as ceramide trihexoside , CD77 (as a receptor), Gb3 or GL-3 , is the name for a group of gangliosides that are components of the cell membrane .

description

The globotriaosylceramides belong to the glycolipid family and consist of a globotriose molecule (α-D-Gal- (1 → 4) -β-D-Gal- (1 → 4) -D-Glc) which is β-glycosidic to a ceramide is bound. The only difference between the globotriaosylceramides is the different ceramide groups, indicated by an 'R' in the structural formula.

In the cells Gb3 of galactose and lactosylceramide under the catalytic influence of the enzyme α-1,4-galactosyltransferase , which in humans by the A4GALT - gene encoded , is produced. Globotriaosylceramide is broken down by the lysosomal enzyme α-galactosidase A ( GAL gene in humans). A deficiency of α-galactosidase A leads to the accumulation of globotriaosylceramide in the lysosome, which leads to the lysosomal storage disease Fabry disease in humans .

function

Gb3 acts as a non- peptide receptor on the cell membrane . Among other things, the Shiga toxin binds to Gb3. It is then transported into the cell by means of endocytosis . In some cancers , Gb3 is overexpressed on the cell membrane . Gb3 could therefore be a target for future cancer therapies.

Individual evidence

  1. ^ Karl-Erik Falk, Karl-Anders Karlsson, Bo E. Samuelsson: Proton nuclear magnetic resonance analysis of anomeric structure of glycosphingolipids . In: Archives of Biochemistry and Biophysics . tape 192 , no. 1 , January 1, 1979, p. 164–176 , doi : 10.1016 / 0003-9861 (79) 90082-1 ( sciencedirect.com [accessed October 5, 2016]).
  2. ↑ Globotriaosylceramide.  In: Online Mendelian Inheritance in Man . (English)
  3. ^ RO Brady : Fabry Disease - An Overview. In: D. Elstein, G. Altarescu, M. Beck (Eds.): Fabry Disease. Verlag Springer, 2010, ISBN 90-481-9032-0 , p. XIX restricted preview in the Google book search
  4. ^ R. Gerhard, I. Just: Bacterial Enterotoxine In: WF Caspary, M. Kist, J. Stein (Ed.): Infectiology of the gastrointestinal tract. Verlag Springer, 2006, ISBN 3-540-41359-6 , p. 41. Restricted preview in the Google book search
  5. ↑ S. Suerbaum, J. Bockmühl, H. Karch: Enterobacteria. In: H. Hahn, SHE Kaufmann, TF Schulz, S. Suerbaum: Medical microbiology and infectiology. Verlag Springer, 2008, ISBN 3-540-46359-3 , p. 246. Limited preview in the Google book search
  6. ↑ U. Distler, J. Souady et al. a .: Shiga toxin receptor Gb3Cer / CD77: tumor association and promising therapeutic target in pancreas and colon cancer. In: PloS one. Volume 4, number 8, 2009, p. E6813, doi : 10.1371 / journal.pone.0006813 . PMID 19714252 . PMC 2730034 (free full text).