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Merck

G9534

Sigma-Aldrich

Globotriaosylsphingosine from porcine blood

Sinónimos:

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

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About This Item

Fórmula empírica (notación de Hill):
C36H67NO17
Número de CAS:
Peso molecular:
785.91
MDL number:
UNSPSC Code:
12352211
PubChem Substance ID:
NACRES:
NA.25

lipid type

sphingolipids

Quality Level

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

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, type N95 (US)


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Tom G Obrig et al.
Current topics in microbiology and immunology, 357, 105-136 (2011-10-11)
The kidneys are the major organs affected in diarrhea-associated hemolytic uremic syndrome (D(+)HUS). The pathophysiology of renal disease in D(+)HUS is largely the result of the interaction between bacterial virulence factors such as Shiga toxin and lipopolysaccharide and host cells
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)
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)
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
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

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