1-Deoxysphinganine (m18:0)

An atypical sphingolipid

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库存: 现货

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数量
1mg

￥1975.00

1580.00

- +
5mg

￥8800.00

7040.00

- +
10mg

￥15450.00

12360.00

- +

已选 0 种 0 瓶

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货号: ajci21460
CAS: 196497-48-0
别名: ES-285
分子式: C18H39NO
分子量: 285.5
纯度: >98%
溶解度: DMF: 10 mg/ml,DMSO: 2 mg/mL,Ethanol: miscible
储存: Store at -20°C
库存: 现货

Background

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 . 1-Deoxysphinganine is neurotoxic to dorsal root ganglion neurons in vitro, decreasing neurite length and inducing neurite contraction when used at a concentration 1 uM. It is cytotoxic to DU145 cells (IC50 = ~2 uM) but stimulates DNA synthesis in Swiss 3T3 cells when used at a concentration of 1 uM.