Ganglioside Metabolism in Health and Disease

Progress in Molecular Biology and Translational Science
Roger SandhoffKonrad Sandhoff

Abstract

Gangliosides (GGs) are cell type-specific sialic acid-containing glycosphingolipids (GSLs), which are enriched in mammalian brain. Defects in GSL metabolism cause fatal human diseases. GSLs are composed of a hydrophilic oligosaccharide linked in 1-O-position to a hydrophobic ceramide anchor, which itself is composed of a long-chain amino alcohol, the sphingoid base, and an amide-bound acyl chain. Biosynthesis of mammalian GGs and other GSLs starts with the formation of their hydrophobic ceramide anchor in the endoplasmic reticulum, followed by sequential glycosylation reactions along the secretory pathway, mainly at the luminal surface of Golgi and trans-Golgi network (TGN) membranes. Few membrane-anchored and often promiscuous glycosyltransferases allow the formation of cell type-specific glycolipid patterns in a combinatorial process. Inherited defects of these transferases therefore affect not only single structures but defined glycolipid series. GGs and other GSLs are thereafter transported by an exocytotic membrane flow to the plasma membrane where they are expressed in cell type-specific patterns, which can be modified by metabolic reactions at or near the cellular surface. Endocytosed (glyco)sphingolipids are degraded, t...Continue Reading

Citations

May 29, 2018·FEBS Letters·Roger Sandhoff, Konrad Sandhoff
Feb 8, 2020·International Journal of Molecular Sciences·Péter SánthaGábor Jancsó
Jan 22, 2020·Journal of Mass Spectrometry : JMS·Raluca IcaAlina D Zamfir
Jun 28, 2020·FEBS Letters·Maria J SarmentoRadek Šachl
Mar 27, 2020·Critical Reviews in Food Science and Nutrition·Xiaoxu WangChanghu Xue
Aug 29, 2019·Frontiers in Cell and Developmental Biology·Emanuela Felley-Bosco, Lisette Leyton
Feb 6, 2020·International Journal of Molecular Sciences·Elena ChiricozziLaura Mauri
Sep 24, 2020·International Journal of Molecular Sciences·Alex E RyckmanJagdeep S Walia
Jul 10, 2019·International Journal of Molecular Sciences·Rossella Indellicato, Marco Trinchera
Dec 24, 2019·Frontiers in Cell and Developmental Biology·Johannes M F G AertsR G Boot
Mar 27, 2019·Molecular Neurobiology·Elena ChiricozziSandro Sonnino
Apr 11, 2020·International Journal of Molecular Sciences·Bernadette Breiden, Konrad Sandhoff
Dec 20, 2019·Scientific Reports·Elena ChiricozziSandro Sonnino
Mar 10, 2021·Analytical and Bioanalytical Chemistry·Zhucui Li, Qibin Zhang
Jun 30, 2021·Journal of Agricultural and Food Chemistry·Yingxu MaChanghu Xue
Aug 20, 2021·Frontiers in Microbiology·Jian WangNan Yao
Nov 20, 2021·Biophysical Journal·Maria J SarmentoRadek Šachl

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