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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

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Certificates of Analysis (COA)

Lot/Batch Number
MKCV1869
MKCT4346
MKCR7530
MKCR4338
MKCQ5619

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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))
F O Dupont et al.
Current medicinal chemistry, 20(2), 280-288 (2012-10-25)
Fabry disease is an X-linked, multisystemic lysosomal storage disorder due to alpha-galactosidase A deficiency. It is characterized by the accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb(3)), in biological fluids, vascular endothelium, heart, and kidneys. Treatment by enzyme replacement therapy has been
Bouwien E Smid et al.
Orphanet journal of rare diseases, 6, 69-69 (2011-11-02)
Enzyme replacement therapy is currently the only approved therapy for Fabry disease. From June 2009 on, viral contamination of Genzyme's production facility resulted in a worldwide shortage of agalsidase beta leading to involuntary dose reductions (approved dose 1 mg/kg/eow, reduced
Saskia M Rombach et al.
Hypertension (Dallas, Tex. : 1979), 60(4), 998-1005 (2012-08-08)
Fabry disease is an X-linked hereditary lysosomal storage disorder attributed to a deficiency of α-galactosidase A leading to increased plasma levels of globotriaosylsphingosine (lysoGb3). The disease presents as a vascular disease, with cerebral, cardiac, and renal complications. Carotid intima-media thickness
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