Chemistry:Psychosine
From HandWiki
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
The enzyme sphingosine beta-galactosyltransferase converts sphingosine to psychosine:
- REDIRECT Template:Chemical reaction
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
- ↑ 1.0 1.1 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. 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. 68. pp. 115–126. doi:10.1007/978-1-4684-7735-1_8. ISBN 978-1-4684-7737-5.
- ↑ 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. doi:10.1177/17590914221087817. 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.
 |  |
