形狀
powder
包裝
pkg of 1 × 1 mg (860476P-1mg)
製造商／商標名
Avanti Research™ - A Croda Brand 860476P
運輸包裝
dry ice
儲存溫度
−20°C
SMILES 字串
O[C@](CN)([H])CCCCCCCCCCCCCC(C([2H])([2H])[2H])([2H])[2H]
一般說明
Desoxymethylsphinganine is a are the major sphingoid-base present in mammals.
應用
1-desoxymethylsphinganine-d5 may be used as an internal standard for the sphingoid analysis from plasma and tissue.
生化／生理作用
Mutations in the palmitoyltransferase (SPT) in hereditary sensory neuropathy type 1 (HSAN-1), due to altered substrate specificity 1-desoxymethylsphinganine and 1-deoxy-sphinganine.
包裝
5 mL Amber Glass Screw Cap Vial (860476P-1mg)
法律資訊
Avanti Research is a trademark of Avanti Polar Lipids, LLC
儲存類別代碼
11 - Combustible Solids
水污染物質分類（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)
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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