Psychosine
 | |
| Names | |
|---|---|
| IUPAC name
(2S,3R,4E)-2-Amino-3-hydroxyoctadec-4-en-1-yl β-D-galactopyranoside
| |
| Systematic IUPAC name
(2R,3R,4S,5R,6R)-2-{[(2S,3R,4E)-2-Amino-3-hydroxyoctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxane-3,4,5-triol | |
| Identifiers | |
3D model (JSmol)
|
|
| 52571 | |
| ChEBI | |
| ChemSpider | |
| ECHA InfoCard | 100.164.357 |
| EC Number |
|
| KEGG | |
PubChem CID
|
|
CompTox Dashboard (EPA)
|
|
| |
| |
| Properties | |
| C24H47NO7 | |
| Molar mass | 461.640 g·mol−1 |
| Hazards | |
| GHS labelling: | |
| |
| Warning | |
| H302, H312, H332 | |
| P261, P264, P270, P271, P280, P301+P312, P302+P352, P304+P312, P304+P340, P312, P322, P330, P363, P501 | |
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
| |
Psychosine is a highly cytotoxic lipid that accumulates in the nervous system in the absence of galactosylceramidase and has been associated with Krabbe disease.[1][2][3]
Formation
[edit]The enzyme sphingosine beta-galactosyltransferase converts sphingosine to psychosine:
In this reaction, a galactose sugar from uridine diphosphate galactose is added to sphingosine at its terminal hydroxy group. Uridine diphosphate (UDP) is the byproduct.[1][4]
References
[edit]- ^ a b Hawkins-Salsbury, J. A.; Parameswar, A. R.; Jiang, X; Schlesinger, P. H.; Bongarzone, E; Ory, D. S.; Demchenko, A. V.; Sands, M. S. (2013). "Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture". The Journal of Lipid Research. 54 (12): 3303–3311. doi:10.1194/jlr.M039610. PMC 3826678. PMID 24006512.
- ^ Vanier, Marie-Thérèse; Svennerholm, Lars (1976). "Chemical Pathology of Krabbe Disease: The Occurrence of Psychosine and Other Neutral Sphingoglycolipids". Current Trends in Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology. Vol. 68. pp. 115–126. doi:10.1007/978-1-4684-7735-1_8. ISBN 978-1-4684-7737-5. PMID 937104.
- ^ Reiter, Cory R.; Rebiai, Rima; Kwak, Angelika; et al. (2022). "The Pathogenic Sphingolipid Psychosine is Secreted in Extracellular Vesicles in the Brain of a Mouse Model of Krabbe Disease". Asn Neuro. 14 17590914221087817. doi:10.1177/17590914221087817. PMC 8943320. PMID 35300522.
- ^ Cleland, W.W.; Kennedy, Eugene P. (1960). "The Enzymatic Synthesis of Psychosine". Journal of Biological Chemistry. 235: 45–51. doi:10.1016/S0021-9258(18)69582-1. PMID 13810623.