An atypical sphingolipid; plasma levels of 1-deoxysphinganine are increased in patients with HSAN1 and GSDI; not converted to canonical sphingolipids or fatty acids and accumulates in mitochondria

1-Deoxysphinganine (m18:0) – 5 mg

Brand:
Cayman
CAS:
196497-48-0
Storage:
-20
UN-No:
Non-Hazardous - /

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.{17311} 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).{42288,36918} 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.{42285} 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 (Item No. 62580).{17311} 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.{42288} 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.{17311,36917}  

 

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Description

An atypical sphingolipid; plasma levels of 1-deoxysphinganine are increased in patients with HSAN1 and GSDI; not converted to canonical sphingolipids or fatty acids and accumulates in mitochondria, inducing mitochondrial fragmentation and dysfunction; accumulates in LLC-PK1 cells and in mouse liver and kidney following application or administration, respectively, of fumonison B1; neurotoxic to dorsal root ganglion neurons in vitro at 1 µM; cytotoxic to DU145 cells (IC50 = ~2 µM) but stimulates DNA synthesis in Swiss 3T3 cells at 1 µM

Formal name: 2S-amino-3R-octadecanol

Synonyms:  1-deoxySA|ES-285|Spisulosine

Molecular weight: 285.5

CAS: 196497-48-0

Purity: ≥95%

Formulation: A crystalline solid

Product Type|Biochemicals|Lipids|Sphingolipids||Research Area|Endocrinology & Metabolism|Carbohydrate Metabolism||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Lipid Biochemistry|Sphingolipids

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