| Record Information |
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| Version | 4.0 |
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| Status | Expected but not Quantified |
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| Creation Date | 2005-11-16 15:48:42 UTC |
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| Update Date | 2020-04-23 20:56:01 UTC |
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| HMDB ID | HMDB0000596 |
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| Secondary Accession Numbers | |
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| Metabolite Identification |
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| Common Name | Glucosylsphingosine |
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| Description | Glucosylsphingosine, also known as glucopsychosine or GLCSPH, belongs to the class of organic compounds known as glycosphingolipids. These are sphingolipids containing a saccharide moiety glycosidically attached to the sphingoid base. Although saccharide moieties are mostly O-glycosidically linked to the ceramide moiety, other sphingolipids with glycosidic bonds of other types (e.g. S-,C-, or N-type) has been reported. Glucosylsphingosine is a very strong basic compound (based on its pKa). |
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| Structure | |
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| Synonyms | | Value | Source |
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| (R-(R*,s*-(e)))-beta-D-glucopyranoside 2-amino-3-hydroxy-4-octadecenyl | HMDB | | (R-(R*,s*-(e)))-beta-delta-glucopyranoside 2-amino-3-hydroxy-4-octadecenyl | HMDB | | D-Glucosyl-N-acylsphingosine | HMDB | | delta-Glucosyl-N-acylsphingosine | HMDB | | Glucopsychosine | HMDB | | Glucosyl psychosine | HMDB | | Glucosyl sphingosine | HMDB | | Sphingosyl beta-glucoside | HMDB | | GLCSPH | HMDB | | Glucosylsphingosine | MeSH |
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| Chemical Formula | C24H47NO7 |
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| Average Molecular Weight | 461.6325 |
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| Monoisotopic Molecular Weight | 461.335252863 |
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| IUPAC Name | (3R,4S,5S,6R)-2-{[(4E)-2-amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol |
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| Traditional Name | glucopsychosine |
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| CAS Registry Number | 52050-17-6 |
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| SMILES | CCCCCCCCCCCCC\C=C\C(O)C(N)COC1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O |
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| InChI Identifier | InChI=1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18?,19?,20-,21-,22+,23-,24?/m1/s1 |
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| InChI Key | HHJTWTPUPVQKNA-KEVGHMQTSA-N |
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| Chemical Taxonomy |
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| Description | belongs to the class of organic compounds known as glycosphingolipids. These are sphingolipids containing a saccharide moiety glycosidically attached to the sphingoid base. Although saccharide moieties are mostly O-glycosidically linked to the ceramide moiety, other sphingolipids with glycosidic bonds of other types (e.g. S-,C-, or N-type) has been reported. |
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| Kingdom | Organic compounds |
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| Super Class | Lipids and lipid-like molecules |
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| Class | Sphingolipids |
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| Sub Class | Glycosphingolipids |
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| Direct Parent | Glycosphingolipids |
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| Alternative Parents | |
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| Substituents | - Glycosphingolipid
- Fatty acyl glycoside
- Fatty acyl glycoside of mono- or disaccharide
- Alkyl glycoside
- Hexose monosaccharide
- Glycosyl compound
- O-glycosyl compound
- Fatty acyl
- Oxane
- Monosaccharide
- Secondary alcohol
- Oxacycle
- Organoheterocyclic compound
- Acetal
- Polyol
- Organic oxygen compound
- Organonitrogen compound
- Organooxygen compound
- Primary aliphatic amine
- Primary alcohol
- Primary amine
- Alcohol
- Organic nitrogen compound
- Amine
- Hydrocarbon derivative
- Organopnictogen compound
- Aliphatic heteromonocyclic compound
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| Molecular Framework | Aliphatic heteromonocyclic compounds |
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| External Descriptors | Not Available |
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| Ontology |
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| Physiological effect | Organoleptic effect: |
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| Disposition | Route of exposure: Source: Biological location: |
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| Process | Naturally occurring process: |
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| Role | Industrial application: Biological role: |
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| Physical Properties |
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| State | Solid |
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| Experimental Properties | | Property | Value | Reference |
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| Melting Point | Not Available | Not Available | | Boiling Point | Not Available | Not Available | | Water Solubility | Not Available | Not Available | | LogP | Not Available | Not Available |
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| Predicted Properties | |
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| General References | - Kaloterakis A, Filiotou A, Koskinas J, Raptis I, Zouboulis C, Michelakakis H, Hadziyannis S: Systemic AL amyloidosis in Gaucher disease. A case report and review of the literature. J Intern Med. 1999 Dec;246(6):587-90. [PubMed:10620102 ]
- Beutler E: Gaucher disease. Blood Rev. 1988 Mar;2(1):59-70. [PubMed:3289655 ]
- Kaye EM, Ullman MD, Wilson ER, Barranger JA: Type 2 and type 3 Gaucher disease: a morphological and biochemical study. Ann Neurol. 1986 Aug;20(2):223-30. [PubMed:3752966 ]
- Holleran WM, Ginns EI, Menon GK, Grundmann JU, Fartasch M, McKinney CE, Elias PM, Sidransky E: Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease. J Clin Invest. 1994 Apr;93(4):1756-64. [PubMed:8163674 ]
- Conradi NG, Kalimo H, Sourander P: Reactions of vessel walls and brain parenchyma to the accumulation of Gaucher cells in the Norrbottnian type (type III) of Gaucher disease. Acta Neuropathol. 1988;75(4):385-90. [PubMed:3364161 ]
- Smith RL, Hutchins GM, Sack GH Jr, Ridolfi RL: Unusual cardiac, renal and pulmonary involvement in Gaucher's disease. Intersitial glucocerebroside accumulation, pulmonary hypertension and fatal bone marrow embolization. Am J Med. 1978 Aug;65(2):352-60. [PubMed:686020 ]
- Alessandrini F, Pfister S, Kremmer E, Gerber JK, Ring J, Behrendt H: Alterations of glucosylceramide-beta-glucosidase levels in the skin of patients with psoriasis vulgaris. J Invest Dermatol. 2004 Dec;123(6):1030-6. [PubMed:15610510 ]
- Ohashi T: [Gene therapy for Gaucher disease]. Nihon Rinsho. 1995 Dec;53(12):3089-94. [PubMed:8577064 ]
- Nishimura RN, Barranger JA: Neurologic complications of Gaucher's disease, type 3. Arch Neurol. 1980 Feb;37(2):92-3. [PubMed:6766716 ]
- Eto Y, Ida H: [Molecular studies of Gaucher disease]. Rinsho Byori. 1996 Apr;44(4):327-34. [PubMed:8847814 ]
- Naito M, Takahashi K, Hojo H: An ultrastructural and experimental study on the development of tubular structures in the lysosomes of Gaucher cells. Lab Invest. 1988 May;58(5):590-8. [PubMed:3367638 ]
- Mariani G, Filocamo M, Giona F, Villa G, Amendola A, Erba P, Buffoni F, Copello F, Pierini A, Minichilli F, Gatti R, Brady RO: Severity of bone marrow involvement in patients with Gaucher's disease evaluated by scintigraphy with 99mTc-sestamibi. J Nucl Med. 2003 Aug;44(8):1253-62. [PubMed:12902415 ]
- Soffer D, Yamanaka T, Wenger DA, Suzuki K, Suzuki K: Central nervous system involvement in adult-onset Gaucher's disease. Acta Neuropathol. 1980;49(1):1-6. [PubMed:7355669 ]
- Deguchi H, Fernandez JA, Pabinger I, Heit JA, Griffin JH: Plasma glucosylceramide deficiency as potential risk factor for venous thrombosis and modulator of anticoagulant protein C pathway. Blood. 2001 Apr 1;97(7):1907-14. [PubMed:11264150 ]
- Ohashi T: [Gaucher disease]. Nihon Rinsho. 1995 Dec;53(12):2943-6. [PubMed:8577040 ]
- Starzl TE, Demetris AJ, Trucco M, Ricordi C, Ildstad S, Terasaki PI, Murase N, Kendall RS, Kocova M, Rudert WA, et al.: Chimerism after liver transplantation for type IV glycogen storage disease and type 1 Gaucher's disease. N Engl J Med. 1993 Mar 18;328(11):745-9. [PubMed:8437594 ]
- Sillence DJ, Puri V, Marks DL, Butters TD, Dwek RA, Pagano RE, Platt FM: Glucosylceramide modulates membrane traffic along the endocytic pathway. J Lipid Res. 2002 Nov;43(11):1837-45. [PubMed:12401882 ]
- Saito M, Saito M, Cooper TB, Vadasz C: Ethanol-induced changes in the content of triglycerides, ceramides, and glucosylceramides in cultured neurons. Alcohol Clin Exp Res. 2005 Aug;29(8):1374-83. [PubMed:16131844 ]
- Pilz H, Heipertz R: [Differential diagnosis of congenital lipidoses by lipid analyses of body fluids, biopsy and autopsy tissue]. Fortschr Neurol Psychiatr Grenzgeb. 1975 Nov;43(11):602-17. [PubMed:53174 ]
- Nilsson O, Grabowski GA, Ludman MD, Desnick RJ, Svennerholm L: Glycosphingolipid studies of visceral tissues and brain from type 1 Gaucher disease variants. Clin Genet. 1985 May;27(5):443-50. [PubMed:3924448 ]
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