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Academic Journal

The mechanism of glycosphingolipid degradation revealed by a GALC-SapA complex structure.

  • Authors : Hill CH; Cambridge Institute for Medical Research, Department of Pathology, University of Cambridge, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0XY, UK.; MRC Laboratory of Molecular Biology, Francis Crick Avenue, Cambridge Biomedical Campus, Cambridge, CB2 0QH, UK.

Subjects: Galactosylceramidase/Galactosylceramidase/Galactosylceramidase/*metabolism ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism ; Saposins/Saposins/Saposins/*metabolism

  • Source: Nature communications [Nat Commun] 2018 Jan 11; Vol. 9 (1), pp. 151. Date of Electronic Publication: 2018 Jan 11.Publisher: Nature Pub. Group Country of Publication: England NLM ID: 101528555 Publication Model: Electronic Cited Medium: Internet ISSN:

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Academic Journal

Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice.

  • Authors : Sun Y; The Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA. ; Zamzow M

Subjects: Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism ; Nervous System Diseases/Nervous System Diseases/Nervous System Diseases/*metabolism ; Saposins/Saposins/Saposins/*deficiency

  • Source: Human molecular genetics [Hum Mol Genet] 2013 Jun 15; Vol. 22 (12), pp. 2435-50. Date of Electronic Publication: 2013 Feb 27.Publisher: IRL Press at Oxford University Press Country of Publication: England NLM ID: 9208958 Publication Model: Print-Electronic Cited Medium:

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Academic Journal

The lipoprotein receptor-related protein-1 (LRP) adapter protein GULP mediates trafficking of the LRP ligand prosaposin, leading to sphingolipid and free cholesterol accumulation in late endosomes and impaired efflux.

  • Authors : Kiss RS; Lipoprotein and Atherosclerosis Research Group, Department of Pathology and Laboratory Medicine, University of Ottawa Heart Institute, Ottawa K1Y 4W7, Canada.; Ma Z

Subjects: Adaptor Proteins, Signal Transducing/Adaptor Proteins, Signal Transducing/Adaptor Proteins, Signal Transducing/*metabolism ; Cholesterol/Cholesterol/Cholesterol/*metabolism ; Endosomes/Endosomes/Endosomes/*metabolism

  • Source: The Journal of biological chemistry [J Biol Chem] 2006 Apr 28; Vol. 281 (17), pp. 12081-92. Date of Electronic Publication: 2006 Feb 22.Publisher: Elsevier Inc. on behalf of American Society for Biochemistry and Molecular Biology Country of Publication: United States NLM ID: 2985121R Publication

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Academic Journal

Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice.

  • Authors : Sun Y; Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA.; Witte DP

Subjects: Brain/Brain/Brain/*metabolism ; Glucosylceramidase/Glucosylceramidase/Glucosylceramidase/*metabolism ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism

  • Source: Human molecular genetics [Hum Mol Genet] 2008 Aug 01; Vol. 17 (15), pp. 2345-56. Date of Electronic Publication: 2008 May 14.Publisher: IRL Press at Oxford University Press Country of Publication: England NLM ID: 9208958 Publication Model: Print-Electronic Cited Medium:

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Academic Journal

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

  • Authors : Sandhoff R; Lipid Pathobiochemistry Group, German Cancer Research Center, Heidelberg, Germany.; Sandhoff K

Subjects: Glycosphingolipids*/Glycosphingolipids*/Glycosphingolipids*/chemistry ; Glycosphingolipids*/Glycosphingolipids*/Glycosphingolipids*/metabolism ; Lysosomal Storage Diseases*/Lysosomal Storage Diseases*/Lysosomal Storage Diseases*/genetics

  • Source: Advances in neurobiology [Adv Neurobiol] 2023; Vol. 29, pp. 333-390.Publisher: Springer Country of Publication: United States NLM ID: 101571545 Publication Model: Print Cited Medium: Print ISSN: 2190-5215 (Print)

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Academic Journal

Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts.

  • Authors : Sadeghlar F; Kekulé-Institut für Organische Chemie und Biochemie der Universität Bonn, Gerhard-Domagk-Strasse 1, 53121 Bonn, Germany.; Remmel N

Subjects: Glycoproteins/Glycoproteins/Glycoproteins/*metabolism ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism; Base Sequence

  • Source: Biochimie [Biochimie] 2003 Mar-Apr; Vol. 85 (3-4), pp. 439-48.Publisher: Editions Scientifiques Elsevier Country of Publication: France NLM ID: 1264604 Publication Model: Print Cited Medium: Print ISSN:

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Academic Journal

Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency.

  • Authors : Potratz A; Kekulé-Institut für Organische Chemie und Biochemie, Bonn, Germany.; Hüttler S

Subjects: Gangliosidoses, GM2/Gangliosidoses, GM2/Gangliosidoses, GM2/*metabolism ; Glycoproteins/Glycoproteins/Glycoproteins/*genetics ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism

  • Source: Biochimica et biophysica acta [Biochim Biophys Acta] 2000 Nov 15; Vol. 1502 (3), pp. 391-7.Publisher: Elsevier Pub. Co Country of Publication: Netherlands NLM ID: 0217513 Publication Model: Print Cited Medium: Print ISSN: 0006-3002

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Academic Journal

Sphingolipid hydrolases and activator proteins.

  • Authors : Bierfreund U; Kekulé-Institut für Organische Chemie und Biochemie, Universität Bonn, Germany.; Kolter T

Subjects: Enzyme Activators/Enzyme Activators/Enzyme Activators/*metabolism ; Glycoproteins/Glycoproteins/Glycoproteins/*metabolism ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism

  • Source: Methods in enzymology [Methods Enzymol] 2000; Vol. 311, pp. 255-76.Publisher: Academic Press Country of Publication: United States NLM ID: 0212271 Publication Model: Print Cited Medium: Print ISSN: 0076-6879

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Academic Journal

Processing of sphingolipid activator proteins and the topology of lysosomal digestion.

  • Authors : Sandhoff K; Kekulé-Institut für Organische Chemie und Biochemie der Universität, Bonn, Germany. ; Kolter T

Subjects: Glycoproteins/Glycoproteins/Glycoproteins/*metabolism ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism ; Lysosomal Storage Diseases/Lysosomal Storage Diseases/Lysosomal Storage Diseases/*metabolism

  • Source: Acta biochimica Polonica [Acta Biochim Pol] 1998; Vol. 45 (2), pp. 373-84.Publisher: Frontiers Media S.A Country of Publication: Switzerland NLM ID: 14520300R Publication Model: Print Cited Medium: Print ISSN: 0001-527X

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Academic Journal

Accumulation of sphingolipids in SAP-precursor (prosaposin)-deficient fibroblasts occurs as intralysosomal membrane structures and can be completely reversed by treatment with human SAP-precursor.

Subjects: Glycoproteins/Glycoproteins/Glycoproteins/*pharmacology ; Glycosphingolipids/Glycosphingolipids/Glycosphingolipids/*metabolism ; Intracellular Membranes/Intracellular Membranes/Intracellular Membranes/*metabolism

  • Source: European journal of cell biology [Eur J Cell Biol] 1997 May; Vol. 73 (1), pp. 10-8.Publisher: Elsevier Country of Publication: Germany NLM ID: 7906240 Publication Model: Print Cited Medium: Print ISSN: 0171-9335 (Print)

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  • 1-10 of  23 results for ""SAPOSINS""