860636P
Avanti
葡糖基(β) 鞘氨醇-d5
Avanti Research™ - A Croda Brand
别名:
D-葡萄糖基-β-1,1′-D-赤-鞘氨醇-d5
表单
powder
包装
pkg of 1 × 1 mg (860636P-1mg)
制造商/商品名称
Avanti Research™ - A Croda Brand
运输
dry ice
储存温度
−20°C
SMILES字符串
[H][C@](/C=C/CCCCCCCCCCCC([2H])(C([2H])([2H])[2H])[2H])(O)[C@](N)([H])CO[C@H](O1)[C@H](O)[C@@H](O)[C@@H]([C@H]1CO)O
InChI
1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18-,19+,20+,21+,22-,23+,24+/m0/s1/i1D3,2D2
InChI key
HHJTWTPUPVQKNA-JCMYXXMQSA-N
应用
葡萄糖基（β）鞘氨醇-d5已用作内标，用于通过液相色谱-串联质谱法定量各种样品中的溶血己糖基神经酰胺。
生化/生理作用
葡萄糖基鞘氨醇具有调节脑微粒体中Ca2+释放的能力。它作为戈谢病（GD）的潜在生物标记物。
包装
5 mL棕色玻璃螺旋盖小瓶（860636P-1mg）
法律信息
Avanti Research is a trademark of Avanti Polar Lipids, LLC
储存分类代码
11 - Combustible Solids
法规信息
美国出口管控产品
Mercedes Roca-Espiau et al.
Journal of orthopaedic surgery and research, 14(1), 383-383 (2019-11-23)
Chronic fatigue (CFg) is a prevalent symptom in Gaucher disease (GD) at diagnosis (79%) and remains in a quarter of patients after years of therapy. Bone abnormalities are present in over 70% and peripheral neuropathy in about 11% of the
LC-MS/MS multiplex analysis of lysosphingolipids in plasma and amniotic fluid: a novel tool for the screening of sphingolipidoses and Niemann-Pick type C disease
Pettazzoni M, et al.
PLoS ONE, 12(7) (2017)
Glucosylsphingosine is a highly sensitive and specific biomarker for primary diagnostic and follow-up monitoring in Gaucher disease in a non-Jewish, Caucasian cohort of Gaucher disease patients
Rolfs A, et al.
PLoS ONE, 8(11) (2013)
LC-MS/MS multiplex analysis of lysosphingolipids in plasma and amniotic fluid: a novel tool for the screening of sphingolipidoses and Niemann-Pick type C disease
Pettazzoni M, et al.
Testing, 12(7) (2017)
Arndt Rolfs et al.
PloS one, 8(11), e79732-e79732 (2013-11-28)
Gaucher disease (GD) is the most common lysosomal storage disorder (LSD). Based on a deficient β-glucocerebrosidase it leads to an accumulation of glucosylceramide. Standard diagnostic procedures include measurement of enzyme activity, genetic testing as well as analysis of chitotriosidase and
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