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CN

G9534

Globotriaosylsphingosine from porcine blood

Synonym(s):

α-D-Gal-(1→4)-β-D-Gal-(1→4)-β-D-Glc-1→O-sphingosine

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About This Item

Empirical Formula (Hill Notation):
C36H67NO17
CAS Number:
126550-86-5
Molecular Weight:
785.91
NACRES:
NA.25
PubChem Substance ID:
24895378
UNSPSC Code:
12352211
MDL number:
MFCD00675433

Key Documents

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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

100

Related Categories

Storage Class

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, type N95 (US)

Regulatory Information

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Certificates of Analysis (COA)

Lot/Batch Number
MKCQ5619
MKCV1869
MKCT4346
MKCR7530
MKCR4338

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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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