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G9534

Sigma-Aldrich

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
MDL number:
MFCD00675433
UNSPSC Code:
12352211
PubChem Substance ID:
24895378
NACRES:
NA.25

lipid type

sphingolipids

Quality Level

100

storage temp.

−20°C

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

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+

InChI key

GRGNVOCPFLXGDQ-CCEZHUSRSA-N

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Ralf Krüger et al.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 883-884, 128-135 (2011-12-06)
Morbus Fabry is a hereditary metabolic disorder with low prevalence and late clinical manifestation. A defect in the α-galactosidase gene leads to lysosomal accumulation of the glycolipid globotriaosylceramide (Gb3). Gb3 may be used for monitoring of enzyme replacement therapy (ERT)
S M Rombach et al.
Biochimica et biophysica acta, 1802(9), 741-748 (2010-05-18)
Fabry disease is an X-linked lysosomal storage disorder due to deficiency of alpha-Galactosidase A, causing accumulation of globotriaosylceramide and elevated plasma globotriaosylsphingosine (lysoGb3). The diagnostic value and clinical relevance of plasma lysoGb3 concentration was investigated. All male and adult female
Early cerebral manifestations in a young female with Fabry disease with skewed X-inactivation.
M G Bouwman et al.
Clinical genetics, 80(5), 500-502 (2012-01-17)
Abdessamad El Alaoui et al.
ChemMedChem, 3(11), 1687-1695 (2008-10-11)
Peripheral benzodiazepine receptors are potential targets for cancer therapeutics through the use of specific ligands such as the pro-apoptotic benzodiazepine RO5-4864. However, the poor water solubility of this compound has been a limitation to its application in vivo. Herein we
Maria D Sanchez-Niño et al.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 26(6), 1797-1802 (2010-05-28)
Transforming growth factor-β1 (TGF-β1) and the macrophage inhibitory factor receptor CD74 link the metabolic disorder with tissue injury in diabetic nephropathy. Fabry disease is an X-linked lysosomal glycosphingolipid storage disorder resulting from a deficient activity of α-galactosidase A that leads
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