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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
产品名称
1-desoxymethylsphinganine-d5, 1-desoxymethylsphinganine-d5 (m17:0), powder
InChI key
UGVBFHUWZNNKIK-RPRHKWDUSA-N
SMILES string
O[C@](CN)([H])CCCCCCCCCCCCCC(C([2H])([2H])[2H])([2H])[2H]
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
Packaging
5 mL Amber Glass Screw Cap Vial (860476P-1mg)
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.
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)
Gergely Karsai et al.
The Journal of biological chemistry, 295(7), 1889-1897 (2019-12-22)
Sphingolipids (SLs) are structurally diverse lipids that are defined by the presence of a long-chain base (LCB) backbone. Typically, LCBs contain a single Δ4E double bond (DB) (mostly d18:1), whereas the dienic LCB sphingadienine (d18:2) contains a second DB at
Biophysical properties of novel 1-deoxy-(dihydro) ceramides occurring in mammalian cells
Jimenez-Rojo N, et al.
Biophysical Journal, 107(12), 2850-2859 (2014)
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)
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
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