Accession NumberHMDB06598
Common_NameIsoglobotriaose
DescriptionIsoglobotriaose is a glycosphingolipid found in mammalian tissues; nonepithelial residue in the intestine, spleen, and spleen microsomes. (PMID: 7053385, 893368, 4690582) Globotriaosylceramide receptors bind to Verotoxin 1 (VT1) B subunits from Escherichia coli eliciting human vascular disease as a consequence of specific binding to these receptors on endothelial cell surfaces. (PMID: 10361298, 10353828) Globotriaosylceramides accumulate in patients with Fabry disease. Fabry disease is a X-linked metabolic disorder caused by a deficiency of alpha-galactosidase leading to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties; glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. (PMID: 10339603)
Chemical_IUPAC_NameNot Available
Chemical FormulaC19H34O15
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