G9534
Globotriaosylsphingosine from porcine blood
别名:
α-D-Gal-(1→4)-β-D-Gal-(1→4)-β-D-Glc-1→O-sphingosine
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关于此项目
经验公式（希尔记法）:
C36H67NO17
化学文摘社编号:
分子量:
785.91
NACRES:
NA.25
PubChem Substance ID:
UNSPSC Code:
12352211
MDL number:
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+
SMILES string
CCCCCCCCCCCCC\C=C\C(O)C(N)COC1OC(CO)C(OC2OC(CO)C(OC3OC(CO)C(O)C(O)C3O)C(O)C2O)C(O)C1O
InChI key
GRGNVOCPFLXGDQ-CCEZHUSRSA-N
lipid type
sphingolipids
storage temp.
−20°C
Quality Level
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, type N95 (US)
法规信息
涉药品监管产品
此项目有
Michel Boutin et al.
Clinica chimica acta; international journal of clinical chemistry, 414, 273-280 (2012-10-09)
Fabry disease is a complex, multisystemic and clinically heterogeneous disease, with elevated excretion of globotriaosylceramide (Gb(3)) and globotriaosylsphingosine (lyso-Gb(3)) accumulating in biological fluids caused by deficiency of the enzyme, lysosomal α-galactosidase A. Our aims were to propose a tandem mass
Shigeki Sugawara et al.
Biochimica et biophysica acta, 1790(2), 101-109 (2008-11-18)
Heat shock proteins (HSPs) are divided into stress-inducible and constitutive types. Generally, HSP70 (stress inducible) and HSC70 (constitutive) are representative of their types, respectively. From the results of immunocytochemical analysis, both HSP70 and HSC70 were constitutively expressed in globotriaosylceramide (Gb3)-expressing
Henrik Gold et al.
Clinical chemistry, 59(3), 547-556 (2012-12-15)
Biochemical markers that accurately reflect the severity and progression of disease in patients with Fabry disease and their response to treatment are urgently needed. Globotriaosylsphingosine, also called lysoglobotriaosylceramide (lysoGb3), is a promising candidate biomarker. We synthesized lysoGb3 and isotope-labeled [5,6,7,8,9]
Christiane Auray-Blais et al.
Analytical chemistry, 84(6), 2745-2753 (2012-02-09)
Fabry disease is a lysosomal storage disorder caused by deficiency of α-galactosidase A, resulting in glycosphingolipid accumulation in organs and tissues, including plasma and urine. Two disease-specific Fabry biomarkers have been identified and quantified in plasma and urine: globotriaosylceramide (Gb(3))
Mariëlle J van Breemen et al.
Biochimica et biophysica acta, 1812(1), 70-76 (2010-09-21)
Fabry disease is treated by two-weekly infusions with α-galactosidase A, which is deficient in this X-linked globotriaosylceramide (Gb3) storage disorder. Elevated plasma globotriaosylsphingosine (lysoGb3) is a hallmark of classical Fabry disease. We investigated effects of enzyme replacement therapy (ERT) on
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