5020 Galactosylsphingosine Galactosylsphingosine (also known as psychosine), is an intermediate in the biosynthesis of cerebrosides. It is formed from the reaction of sphingosine with UDP-galactose and then reacts with fatty acid-coenzyme A to form the cerebroside. It is a galactoside metabolite of sphingosine and can function as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of galactosylsphingosine are associated with globoid cell leukodystrophy (Krabbe disease), which is characterized by the dysfunction of galactosylceramidase. Galactosylsphingosine is a highly cytotoxic lipid capable of inducing cell death in a wide variety of cell types including oligodendrocytes. It is known to accumulate in the nervous system in the absence of galactosylceramidase. Galactosylsphingosine localizes to lipid rafts and perturbs membrane integrity. It also inhibits protein kinase C translocation to the plasma membrane (PMID: 24006512). Symptoms of Krabbe disease begin between the ages of 3 and 6 months with irritability, fevers, limb stiffness, seizures, feeding difficulties, vomiting, and slowing of mental and motor development. In the first stages of the disease, the symptoms are often mistaken with those of cerebral palsy. Other symptoms include muscle weakness, spasticity, deafness, optic atrophy, optic nerve enlargement, blindness, paralysis, and difficulty when swallowing. 2238-90-6 22833541 C24H47NO7 White powder. No indication of carcinogenicity to humans (not listed by IARC). Chronically high levels of psychosine are associated with Globoid Cell Leukodystrophy. 2014-10-02T18:58:05Z 2018-03-21T17:46:19Z C01747 16874 true CCCCCCCCCCCCC\C=C\C(O)C(N)COC1O[C@H](CO)[C@H](O)[C@@H](O)[C@H]1O C24H47NO7 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 InChIKey=HHJTWTPUPVQKNA-LUIGAKQRSA-N 461.6325 461.335252863 Solid HMDB00648 17215986