Glucosyl sphingosine is a lipid of Sphingolipids (SP) class. Glucosyl sphingosine is associated with abnormalities such as Sphingolipidoses, Globoid cell leukodystrophy, Sandhoff Disease, Tay-Sachs Disease and Fabry Disease. The involved functions are known as Apoptosis, Acetylation, Infiltration, kinase activity and energy pathways. Glucosyl sphingosine often locates in Lysosomal and Protoplasm. The associated genes with Glucosyl sphingosine are MAPT gene, GALC gene, HEXA gene, PICK1 gene and ELF3 gene. The related lipids are lysoganglioside G(M2), Ganglioside GA2, Lysophosphatidylcholines and globotriaosyl lysosphingolipid.
To understand associated biological information of Glucosyl sphingosine, we collected biological information of abnormalities, associated pathways, cellular/molecular locations, biological functions, related genes/proteins, lipids and common seen animal/experimental models with organized paragraphs from literatures.
Glucosyl sphingosine is suspected in Globoid cell leukodystrophy, Sphingolipidoses, Sandhoff Disease, Tay-Sachs Disease, Fabry Disease and other diseases in descending order of the highest number of associated sentences.
| Disease | Cross reference | Weighted score | Related literature |
|---|
We collected disease MeSH terms mapped to the references associated with Glucosyl sphingosine
| MeSH term | MeSH ID | Detail |
|---|---|---|
| Hemolysis | D006461 | 131 associated lipids |
| Multiple Myeloma | D009101 | 13 associated lipids |
| Leukodystrophy, Globoid Cell | D007965 | 6 associated lipids |
| Nerve Degeneration | D009410 | 53 associated lipids |
| Gaucher Disease | D005776 | 13 associated lipids |
| Parkinson Disease | D010300 | 53 associated lipids |
There are no associated biomedical information in the current reference collection.
Associated locations are in red color. Not associated locations are in black.
| Location | Cross reference | Weighted score | Related literatures |
|---|
| Function | Cross reference | Weighted score | Related literatures |
|---|
| Lipid concept | Cross reference | Weighted score | Related literatures |
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| Gene | Cross reference | Weighted score | Related literatures |
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There are no associated biomedical information in the current reference collection.
| Authors | Title | Published | Journal | PubMed Link |
|---|---|---|---|---|
| pmid: | ||||
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| Hara A and Taketomi T | Chemical properties and stereoisomerism of heterogeneous long chain bases in lysosphingolipids by positive ion fast atom bombardment mass spectrometry and carbon-13 NMR spectroscopy. | 1986 | J. Biochem. | pmid:3782058 |
| Yamaguchi Y et al. | The synthetic pathway for glucosylsphingosine in cultured fibroblasts. | 1994 | J. Biochem. | pmid:7852294 |
| Sasagasako N et al. | Glucosylceramide and glucosylsphingosine metabolism in cultured fibroblasts deficient in acid beta-glucosidase activity. | 1994 | J. Biochem. | pmid:8188616 |
| Deguchi H et al. | Sphingolipids as bioactive regulators of thrombin generation. | 2004 | J. Biol. Chem. | pmid:14722105 |
| Sun Y et al. | Ex vivo and in vivo effects of isofagomine on acid β-glucosidase variants and substrate levels in Gaucher disease. | 2012 | J. Biol. Chem. | pmid:22167193 |
| Lloyd-Evans E et al. | Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms. | 2003 | J. Biol. Chem. | pmid:12709427 |
| Gramlich PA et al. | A peptide-linked recombinant glucocerebrosidase for targeted neuronal delivery: Design, production, and assessment. | 2016 | J. Biotechnol. | pmid:26795355 |
| Kanazawa T et al. | Inhibition of cytokinesis by a lipid metabolite, psychosine. | 2000 | J. Cell Biol. | pmid:10811833 |
| Sprong H et al. | Glycosphingolipids are required for sorting melanosomal proteins in the Golgi complex. | 2001 | J. Cell Biol. | pmid:11673476 |
| Imokawa G | A possible mechanism underlying the ceramide deficiency in atopic dermatitis: expression of a deacylase enzyme that cleaves the N-acyl linkage of sphingomyelin and glucosylceramide. | 2009 | J. Dermatol. Sci. | pmid:19443184 |
| Kang L et al. | Successful newborn screening for Gaucher disease using fluorometric assay in China. | 2017 | J. Hum. Genet. | pmid:28356566 |
| Okamoto R et al. | Sphingosylphosphorylcholine is upregulated in the stratum corneum of patients with atopic dermatitis. | 2003 | J. Lipid Res. | pmid:12518027 |
| Sueyoshi N et al. | Apoptosis of Neuro2a cells induced by lysosphingolipids with naturally occurring stereochemical configurations. | 2001 | J. Lipid Res. | pmid:11483620 |
| Sillence DJ et al. | Glucosylceramide modulates membrane traffic along the endocytic pathway. | 2002 | J. Lipid Res. | pmid:12401882 |
| Bodennec J et al. | Simultaneous quantification of lyso-neutral glycosphingolipids and neutral glycosphingolipids by N-acetylation with [3H]acetic anhydride. | 2003 | J. Lipid Res. | pmid:12730305 |
| Gaspar P et al. | Action myoclonus-renal failure syndrome: diagnostic applications of activity-based probes and lipid analysis. | 2014 | J. Lipid Res. | pmid:24212238 |
| Nozawa M et al. | Novel procedure for measuring psychosine derivatives by an HPLC method. | 1992 | J. Neurochem. | pmid:1629732 |
| Taguchi YV et al. | Glucosylsphingosine Promotes α-Synuclein Pathology in Mutant GBA-Associated Parkinson's Disease. | 2017 | J. Neurosci. | pmid:28847804 |
| Pavlova EV et al. | B cell lymphoma and myeloma in murine Gaucher's disease. | 2013 | J. Pathol. | pmid:23775597 |
| Smith NJ et al. | Reduced cerebral vascularization in experimental neuronopathic Gaucher disease. | 2018 | J. Pathol. | pmid:28981147 |
| Mogi C et al. | Sphingosylphosphorylcholine antagonizes proton-sensing ovarian cancer G-protein-coupled receptor 1 (OGR1)-mediated inositol phosphate production and cAMP accumulation. | 2005 | J. Pharmacol. Sci. | pmid:16210776 |
| Ishibashi M et al. | Abnormal expression of the novel epidermal enzyme, glucosylceramide deacylase, and the accumulation of its enzymatic reaction product, glucosylsphingosine, in the skin of patients with atopic dermatitis. | 2003 | Lab. Invest. | pmid:12649340 |
| Igisu H et al. | Inhibition of cytochrome c oxidase and hemolysis caused by lysosphingolipids. | 1988 | Lipids | pmid:2840545 |
| Burrow TA et al. | CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings. | 2015 | Mol. Genet. Metab. | pmid:25219293 |
| Orvisky E et al. | Glucosylsphingosine accumulation in tissues from patients with Gaucher disease: correlation with phenotype and genotype. | 2002 | Mol. Genet. Metab. | pmid:12208131 |
| Tayebi N et al. | Gaucher disease with parkinsonian manifestations: does glucocerebrosidase deficiency contribute to a vulnerability to parkinsonism? | 2003 | Mol. Genet. Metab. | pmid:12809640 |
| Xu YH et al. | Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models. | 2011 | Mol. Genet. Metab. | pmid:21257328 |
| Schueler UH et al. | Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3. | 2003 | Neurobiol. Dis. | pmid:14678774 |