1-Deoxysphinganine (m18:0)

1-Deoxysphinganine is an atypical sphingolipid that lacks the C1-hydroxyl group of canonical sphinganine and is formed when serine palmitoyltransferase condenses palmitoyl-CoA with alanine instead of serine during sphingolipid synthesis. Plasma levels of 1-deoxysphinganine are increased in patients with hereditary sensory and autonomic neuropathy type 1 (HSAN1), an inherited neuropathy associated with serine palmitoyltransferase gene mutations, and in patients with glycogen storage disease type I (GSDI). Deoxysphingolipids, including 1-deoxysphinganine, are not converted to canonical sphingolipids or fatty acids and accumulate in cells, particularly in the mitochondria where 1-deoxysphinganine induces mitochondrial fragmentation and dysfunction. It also accumulates in LLC-PK1 cells and in mouse liver and kidney following application or administration, respectively, of the ceramide synthase inhibitor fumonisin B1 (Cay-62580). 1-Deoxysphinganine is neurotoxic to dorsal root ganglion neurons in vitro, decreasing neurite length and inducing neurite contraction when used at a concentration 1 µM. It is cytotoxic to DU145 cells (IC50 = ~2 µM) but stimulates DNA synthesis in Swiss 3T3 cells when used at a concentration of 1 µM.Formal Name: 2S-amino-3R-octadecanol. CAS Number: 196497-48-0. Synonyms: 1-deoxySA, ES-285, Spisulosine. Molecular Formula: C18H39NO. Formula Weight: 285.5. Purity: >95%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 10 mg/ml, DMSO: 2 mg/ml, Ethanol: miscible. SMILES: CCCCCCCCCCCCCCC[C@@H](O)[C@H](C)N. InChi Code: InChI=1S/C18H39NO/c1-3-4-5-6-7-8-9-10-11-12-13-14-15-16-18(20)17(2)19/h17-18,20H,3-16,19H2,1-2H3/t17-,18+/m0/s1. InChi Key: YRYJJIXWWQLGGV-ZWKOTPCHSA-N.