Biochemistry of glycosphingolipid degradation

Clinica Chimica Acta; International Journal of Clinical Chemistry
Konrad Sandhoff, T Kolter

Abstract

Glycosphingolipids (GSLs) form cell-type-specific patterns on the surface of eukaryotic cells. Degradation of GSLs requires endocytotic membrane flow of plasma membrane-derived GSLs into the lysosomes as the digesting organelles. Recent research focused on the mechanisms leading to selective membrane degradation in the lysosomes and on the mechanism and physiological function of sphingolipid activator proteins, which are needed for degradation of GSLs with short oligosaccharide chains in addition to hydrolysing enzymes. Both, the inherited deficiency of lysosomal hydrolases and of sphingolipid activator proteins give rise to sphingolipid storage diseases. In some cases it was possible to correlate residual enzyme activities with the onset and the course of the disease.

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Citations

May 10, 2005·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·L YarguiA Berhoune
Sep 23, 2000·Chemistry and Physics of Lipids·K FerlinzK Sandhoff
Apr 28, 2001·Prostaglandins & Other Lipid Mediators·S M Mandala
Nov 5, 2003·Gene·Marc J ChampignySuleiman A Igdoura
Oct 9, 2002·Neuropathology and Applied Neurobiology·M JeyakumarF M Platt
Jun 14, 2003·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·Frances M PlattTerry D Butters
Jan 31, 2003·Expert Opinion on Investigational Drugs·Gregory M Pastores, Natalie L Barnett
Jul 8, 2016·International Journal of Molecular Sciences·Maria Francisca CoutinhoSandra Alves
Nov 18, 2017·Nature Reviews. Drug Discovery·Frances M Platt
Jun 2, 2001·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·S PanigoneD Delia
Feb 22, 2002·The Journal of Biological Chemistry·Stephane LefrancoisCarlos R Morales
Mar 12, 2021·Science Translational Medicine·Mandy M T van LeentRaphaël Duivenvoorden
Jun 1, 2000·Biochimica Et Biophysica Acta·A HuwilerK Sandhoff
Oct 17, 2020·Current Pharmaceutical Design·Jayesh Sheth, Aadhira Nair

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