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关于此项目
经验公式（希尔记法）:
C17H32D5NO
化学文摘社编号:
分子量:
276.51
MDL number:
UNSPSC Code:
12352211
NACRES:
NA.25
Assay:
>99% (TLC)
Form:
powder
产品名称
1-desoxymethylsphinganine-d5, 1-desoxymethylsphinganine-d5 (m17:0), powder
assay
>99% (TLC)
form
powder
packaging
pkg of 1 × 1 mg (860476P-1mg)
manufacturer/tradename
Avanti Research™ - A Croda Brand 860476P
shipped in
dry ice
storage temp.
−20°C
SMILES string
O[C@](CN)([H])CCCCCCCCCCCCCC(C([2H])([2H])[2H])([2H])[2H]
InChI key
UGVBFHUWZNNKIK-RPRHKWDUSA-N
General description
Desoxymethylsphinganine is a are the major sphingoid-base present in mammals.
Application
1-desoxymethylsphinganine-d5 may be used as an internal standard for the sphingoid analysis from plasma and tissue.
Biochem/physiol Actions
Mutations in the palmitoyltransferase (SPT) in hereditary sensory neuropathy type 1 (HSAN-1), due to altered substrate specificity 1-desoxymethylsphinganine and 1-deoxy-sphinganine.
Packaging
5 mL Amber Glass Screw Cap Vial (860476P-1mg)
Legal Information
Avanti Research is a trademark of Avanti Polar Lipids, LLC
存储类别
11 - Combustible Solids
wgk
WGK 3
Oral L-serine supplementation reduces production of neurotoxic deoxysphingolipids in mice and humans with hereditary sensory autonomic neuropathy type 1
Garofalo K, et al.
The Journal of Clinical Investigation, 121(12), 4735-4745 (2011)
Annelies Rotthier et al.
Human mutation, 32(6), E2211-E2225 (2011-05-28)
Hereditary sensory and autonomic neuropathy type I (HSAN-I) is an axonal peripheral neuropathy leading to progressive distal sensory loss and severe ulcerations. Mutations in SPTLC1 and SPTLC2, encoding the two subunits of serine palmitoyltransferase (SPT), the enzyme catalyzing the first
Hereditary sensory neuropathy type 1 is caused by the accumulation of two neurotoxic sphingolipids
Penno A, et al.
The Journal of Biological Chemistry, 285(15), 11178-11187 (2010)
全球贸易项目编号
| 货号 | GTIN |
|---|---|
| 860476P-1MG | 04061835191673 |