Psychosine

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 Edit this at Wikidata
EC Number
  • 636-571-3
KEGG
UNII
  • 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+/m0/s1
    Key: HHJTWTPUPVQKNA-PIIMIWFASA-N
  • CCCCCCCCCCCCC/C=C/[C@H]([C@H](CO[C@H]1[C@@H]([C@H]([C@H]([C@H](O1)CO)O)O)O)N)O
Properties
C24H47NO7
Molar mass 461.640 g·mol−1
Hazards
GHS labelling:
GHS07: Exclamation mark
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.

Formation

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.

References

  1. 1 2 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.