ReviewBrain pathology (Zurich, Switzerland)1998
Recent advances in the biochemistry of sphingolipidoses.
Review in Brain pathology (Zurich, Switzerland), 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 87 citations in OpenAlex.
- Selective requirement of glycosphingolipid synthesis for natural killer and cytotoxic T cells.Cell · 2025Article
- Targeted protein degradation: advances in drug discovery and clinical practice.Signal transduction and targeted therapy · 2024Review
- Effects of Gangliosides on Spermatozoa, Oocytes, and Preimplantation Embryos.International journal of molecular sciences · 2019Review
- New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperones.Journal of neuroscience research · 2016Review
- Glycosphingolipid modification: structural diversity, functional and mechanistic integration of diabetes.Diabetes & metabolism journal · 2011Article
- Genetic basis for the lack of N-glycolylneuraminic acid expression in human tissues and its implication to human evolution.Proceedings of the Japan Academy. Series B, Physical and biological sciences · 2006Review
- Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease.Journal of inherited metabolic disease · 2004Article
- Core 2 GlcNAc transferase and kidney tubular cell-specific expression.Glycoconjugate journal · 2004Review
- Future perspectives for glycolipid research in medicine.Philosophical transactions of the Royal Society of London. Series B, Biological sciences · 2003Review
- Migration of nerve growth cones requires detergent-resistant membranes in a spatially defined and substrate-dependent manner.The Journal of cell biology · 2002Article
- Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells.Proceedings of the National Academy of Sciences of the United States of America · 2001Article
- Gaucher disease: understanding the molecular pathogenesis of sphingolipidoses.Journal of inherited metabolic disease · 2001Review
- Cloning and expression of glycolipid transfer protein from bovine and porcine brain.The Journal of biological chemistry · 2000Article
- A non-glycosylated and functionally deficient mutant (N215H) of the sphingolipid activator protein B (SAP-B) in a novel case of metachromatic leukodystrophy (MLD).Journal of inherited metabolic disease · 2000Article
- A vital role for glycosphingolipid synthesis during development and differentiation.Proceedings of the National Academy of Sciences of the United States of America · 1999Article
- A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder.The Journal of clinical investigation · 1999Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Glycosphingolipids are ubiquitous membrane components of eukaryotic cells. They participate in various cell recognition events and can regulate enzymes and receptors within the plasma membrane. Sphingolipidoses are due to an impaired lysosomal digestion of these substances. Glycosphingolipids are degraded by the action of exohydrolases, which are supported, in the case of glycosphingolipids with short oligosaccharide chains, by sphingolipid activator proteins. Five sphingolipid activator proteins are known so far, the GM2-activator and the SAPs, SAP-A to D (also called saposins). Degradation of glycosphingolipids requires endocytic membrane flow of plasma membrane derived glycosphingolipids into the lysosomes. Recent research focused on the topology of this process and on the mechanism and physiological function of sphingolipid activator proteins. Limited knowledge is available about enzymology and topology of glycosphingolipid biosynthesis. Recently, intermediates of this metabolic pathway have been identified as novel signalling molecules. Inhibition of glycosphingolipid biosynthesis has been shown to be beneficial in the animal model of Tay-Sachs disease. Mice with disrupted genes for lysosomal hydrolases and activator proteins are useful models for known human diseases and are valuable tools for the study of glycosphingolipid metabolism, the pathogenesis of sphingolipidoses and novel therapeutic approaches.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.