Psychosine  (Synonyms: Galactosylsphingosine)

Psychosine (Galactosylsphingosine), a substrate of the galactocerebrosidase (GALC) enzyme, is a potential biomarker for Krabbe disease^[1]. Psychosine is a highly cytotoxic lipid, capable of inducing cell death in a wide variety of cell types including, most relevantly to globoid cell leukodystrophy (GLD), oligodendrocytes. Psychosine causes cell death at least in part via apoptosis. Psychosine also is an inhibitor of PKC^[1].

Psychosine, is a substrate of the GALC enzyme that shows promise to aid in the diagnosis and follow-up of at-risk infants identified through newborn screening (NBS)^[1]. Psychosine induces pleiotropic effects, including dysfunctions in several cellular pathways^[2].

MCE has not independently confirmed the accuracy of these methods. They are for reference only.

461.63

Solid

C₂₄H₄₇NO₇

2238-90-6

O[[email protected]]([[email protected]]([[email protected]]([[email protected]@H](CO)O1)O)O)[[email protected]@H]1OC[[email protected]](N)[[email protected]](O)/C=C/CCCCCCCCCCCCC

Room temperature in continental US; may vary elsewhere.

• [1]. M L Escolar, et al. Psychosine, a marker of Krabbe phenotype and treatment effect. Mol Genet Metab. 2017 Jul;121(3):271-278.  [Content Brief]

  [2]. Jacqueline A Hawkins-Salsbury,et al.Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. J Lipid Res.2013 Dec;54(12):3303-11.  [Content Brief]