860476P
Avanti
1-desoxymethylsphinganine-d5
1-desoxymethylsphinganine-d5 (m17:0), powder
Sinónimos:
1-Deoxymethylsphinganine-d5
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About This Item
Fórmula empírica (notación de Hill):
C17H32D5NO
Número de CAS:
Peso molecular:
276.51
UNSPSC Code:
12352211
NACRES:
NA.25
form
powder
packaging
pkg of 1 × 1 mg (860476P-1mg)
manufacturer/tradename
Avanti Polar Lipids 860476P
shipped in
dry ice
storage temp.
−20°C
SMILES string
O[C@](CN)([H])CCCCCCCCCCCCCC(C([2H])([2H])[2H])([2H])[2H]
Categorías relacionadas
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
Storage Class
11 - Combustible Solids
wgk_germany
WGK 3
Certificados de análisis (COA)
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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)
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)
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