Uses
1-Deoxysphingosine is elevated in lymphoblasts of individuals with hereditary sensory neuropathy type 1 (HSAN1) disorder; it features a (4E) double bond in its structure and is produced through the catabolism of 1-deoxyceramide catalyzed by the ceramidase enzyme, classifying it as an unsaturated deoxy-sphingoid base.
References
1. Steiner, R., Saied, E.M., Othman, A., et al. Elucidating the chemical structure of native 1-deoxysphingosine J. Lipid Res. 57(7),1194-1203(2016).
2. Alecu, I., Othman, A., Penno, A., et al. Cytotoxic 1-deoxysphingolipids are metabolized by a cytochrome P450-dependent pathway J. Lipid Res. 58(1),60-71(2017).
3. Hube, L., Dohrn, M.F., Karsai, G., et al. Metabolic syndrome, neurotoxic 1-deoxysphingolipids and nervous tissue inflammation in chronic idiopathic axonal polyneuropathy (CIAP) PLoS One 12(1):e0170583,(2017).