1-Deoxysphing<wbr/>anine (m18:0)

1-Deoxysphinganine (m18:0)

CAT N°: 13511
Price:

From €176.00 €149.60

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}

Territorial Availability: Available through Bertin Technologies only in France

  • Synonyms
    • 2S-amino-3R-octadecanol
  • Correlated keywords
    • C-1 DU-145 3-T-3 LLCPK1 LLCPK-1 B-1 HSAN-1 FA GSD-I ES-285 ES285 deoxy-sphinganine doxSA
  • Product Overview:
    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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