产品名称
Lyso GB3 (synthetic), powder
InChI
1S/C36H67NO17/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-21(41)20(37)19-49-34-30(47)27(44)32(23(17-39)51-34)54-36-31(48)28(45)33(24(18-40)52-36)53-35-29(46)26(43)25(42)22(16-38)50-35/h14-15,20-36,38-48H,2-13,16-19,37H2,1H3/b15-14+/t20-,21+,22+,23+,24+,25-,26-,2
InChI key
GRGNVOCPFLXGDQ-TWHXEDJUSA-N
SMILES string
O[C@H]1[C@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@@H]([C@H](O)[C@H]2O)O[C@H]3O[C@H](CO)[C@@H]([C@H](O)[C@H]3O)O)[C@@H]1O
assay
99% (TLC)
form
powder
packaging
package of 1 × 500 μg (860952P-500UG)
manufacturer/tradename
Avanti Research™ - A Croda Brand
lipid type
sphingolipids
shipped in
dry ice
storage temp.
−20°C
相关类别
General description
Globotriaosylsphingosine (Lyso GB3) is a deacylated form of globotriaosylceramide. It is a circulating bioactive glycolipid.
Application
Globotriaosylsphingosine (Lyso GB3) has been used as a standard in generating calibration curve via ultra-performance liquid chromatography (UPLC) method.
Biochem/physiol Actions
Globotriaosylsphingosine (Lyso GB3) is a potential biomarker of Fabry disease.
Packaging
2 mL Amber Serum Vial with Stopper and Crimp Cap (860952P-500UG)
Legal Information
Avanti Research is a trademark of Avanti Polar Lipids, LLC
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
涉药品监管产品
此项目有
The Fabry disease-associated lipid Lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain
Choi L, et al.
Neuroscience Letters, 594, 163-168 (2015)
Lyso-Gb3 activates Notch1 in human podocytes
Sanchez N, et al.
Human Molecular Genetics, 24(20), 5720-5732 (2015)
Rui Quinta et al.
Gene, 536(1), 97-104 (2013-12-18)
Fabry disease is an X-linked lysosomal storage disease (LSD) caused by deficient activity of α-Galactosidase A (α-Gal A). As a result, glycosphingolipids, mainly globotriaosylceramide (Gb3), progressively accumulate in body fluids and tissues. Studies aiming at the identification of secondary lipid
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