Home > Publications database > Neuronal Ganglioside and Glycosphingolipid (GSL) Metabolism and Disease : Cascades of Secondary Metabolic Errors Can Generate Complex Pathologies (in LSDs). > print

001     182155
005     20240422103653.0
020 _ _ |a 978-3-031-12389-4 (print)
020 _ _ |a 978-3-031-12390-0 (electronic)
024 7 _ |a 10.1007/978-3-031-12390-0_12
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024 7 _ |a pmid:36255681
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024 7 _ |a DOI: 10.1007/978-3-031-12390-0_12
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024 7 _ |a DOI: 10.1007/978-3-031-12390-0_12
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037 _ _ |a DKFZ-2022-02466
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Sandhoff, Roger
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|e First author
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245 _ _ |a Neuronal Ganglioside and Glycosphingolipid (GSL) Metabolism and Disease : Cascades of Secondary Metabolic Errors Can Generate Complex Pathologies (in LSDs).
260 _ _ |a New York, NY
|c 2023
|b Springer
336 7 _ |a article
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336 7 _ |a Output Types/Journal article
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336 7 _ |a Book
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336 7 _ |a ARTICLE
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336 7 _ |a Journal Article
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500 _ _ |a #EA:A411# / Adv Neurobiol = 2190-5223
520 _ _ |a Glycosphingolipids (GSLs) are a diverse group of membrane components occurring mainly on the surfaces of mammalian cells. They and their metabolites have a role in intercellular communication, serving as versatile biochemical signals (Kaltner et al, Biochem J 476(18):2623-2655, 2019) and in many cellular pathways. Anionic GSLs, the sialic acid containing gangliosides (GGs), are essential constituents of neuronal cell surfaces, whereas anionic sulfatides are key components of myelin and myelin forming oligodendrocytes. The stepwise biosynthetic pathways of GSLs occur at and lead along the membranes of organellar surfaces of the secretory pathway. After formation of the hydrophobic ceramide membrane anchor of GSLs at the ER, membrane-spanning glycosyltransferases (GTs) of the Golgi and Trans-Golgi network generate cell type-specific GSL patterns for cellular surfaces. GSLs of the cellular plasma membrane can reach intra-lysosomal, i.e. luminal, vesicles (ILVs) by endocytic pathways for degradation. Soluble glycoproteins, the glycosidases, lipid binding and transfer proteins and acid ceramidase are needed for the lysosomal catabolism of GSLs at ILV-membrane surfaces. Inherited mutations triggering a functional loss of glycosylated lysosomal hydrolases and lipid binding proteins involved in GSL degradation cause a primary lysosomal accumulation of their non-degradable GSL substrates in lysosomal storage diseases (LSDs). Lipid binding proteins, the SAPs, and the various lipids of the ILV-membranes regulate GSL catabolism, but also primary storage compounds such as sphingomyelin (SM), cholesterol (Chol.), or chondroitin sulfate can effectively inhibit catabolic lysosomal pathways of GSLs. This causes cascades of metabolic errors, accumulating secondary lysosomal GSL- and GG- storage that can trigger a complex pathology (Breiden and Sandhoff, Int J Mol Sci 21(7):2566, 2020).
536 _ _ |a 311 - Zellbiologie und Tumorbiologie (POF4-311)
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650 _ 7 |a Alzheimer
|2 Other
650 _ 7 |a Catabolism
|2 Other
650 _ 7 |a Degradation
|2 Other
650 _ 7 |a Development
|2 Other
650 _ 7 |a Endosomal pathway
|2 Other
650 _ 7 |a Frontal lobe dementia
|2 Other
650 _ 7 |a Ganglio-series
|2 Other
650 _ 7 |a Ganglioside
|2 Other
650 _ 7 |a Genetic disease
|2 Other
650 _ 7 |a Glycolipid
|2 Other
650 _ 7 |a Glycosphingolipid
|2 Other
650 _ 7 |a Glycosyltransferase
|2 Other
650 _ 7 |a Hydrolase
|2 Other
650 _ 7 |a Intra-lysosomal luminal vesicle (ILV)
|2 Other
650 _ 7 |a Lysosomal storage disease (LSD)
|2 Other
650 _ 7 |a Lysosome
|2 Other
650 _ 7 |a Membrane-surface
|2 Other
650 _ 7 |a Metabolism
|2 Other
650 _ 7 |a Neurodegenerative disease
|2 Other
650 _ 7 |a Neuron
|2 Other
650 _ 7 |a Organelle
|2 Other
650 _ 7 |a Parkinson
|2 Other
650 _ 7 |a Receptor
|2 Other
650 _ 7 |a Secondary storage
|2 Other
650 _ 7 |a Secretory pathway
|2 Other
650 _ 7 |a Sphingolipid-binding protein (SAP)
|2 Other
650 _ 7 |a Sphingolipid-transfer protein
|2 Other
650 _ 7 |a Topology
|2 Other
700 1 _ |a Sandhoff, Konrad
|b 1
773 _ _ |a DOI: 10.1007/978-3-031-12390-0_12
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|p 333-390
|t Advances in neurobiology
|v 29
|y 2023
|x 2190-5215
909 C O |p VDB
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910 1 _ |a Deutsches Krebsforschungszentrum
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914 1 _ |y 2023
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980 _ _ |a journal
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980 _ _ |a book
980 _ _ |a I:(DE-He78)A411-20160331
980 _ _ |a UNRESTRICTED

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