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@ARTICLE{Sandhoff:136797,
      author       = {R. Sandhoff$^*$ and H. Schulze and K. Sandhoff},
      title        = {{G}anglioside {M}etabolism in {H}ealth and {D}isease.},
      journal      = {G3: Genes, genomes, genetics},
      volume       = {156},
      issn         = {2160-1836},
      address      = {Pittsburgh, PA},
      publisher    = {Genetics Soc. of America},
      reportid     = {DKFZ-2018-01235},
      pages        = {1-62},
      year         = {2018},
      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,
                      together with other membrane lipids in a stepwise fashion by
                      endolysosomal enzymes with the help of small lipid-binding
                      proteins, the sphingolipid activator proteins (SAPs), at the
                      surface of intraluminal lysosomal vesicles. Inherited
                      defects in a sphingolipid-degrading enzyme or SAP cause the
                      accumulation of the corresponding lipid substrates.
                      Endolysosomal GSL degradation is strongly modified by the
                      lipid components of the organelle microenvironments.},
      subtyp        = {Review Article},
      cin          = {G131},
      ddc          = {570},
      cid          = {I:(DE-He78)G131-20160331},
      pnm          = {317 - Translational cancer research (POF3-317)},
      pid          = {G:(DE-HGF)POF3-317},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:29747811},
      doi          = {10.1016/bs.pmbts.2018.01.002},
      url          = {https://inrepo02.dkfz.de/record/136797},
}