Glucosylsphingosine, buttermilk

CATALOG # 1306
Amount 5 mg
Price $330.00
Qty
 
Glucosylsphingosine, buttermilk
  • Catalog #:1306
  • Scientific Name:Glucosylsphingosine, buttermilk
  • Common Name:Glucopsychosine; lyso-Glucocerebroside; 1-beta-D-Glucosylsphingosine
  • Empirical Formula:C24H47NO7
  • CAS#52050-17-6
  • SDSView Safety Data Sheet
  • Data Sheet:View Data Sheet
  • Formula Weight:461
  • Unit:5 mg
  • Solvent:none
  • Source:semisynthetic
  • Purity:98+%
  • Analytical Methods:TLC; identity confirmed by MS
  • Natural Source:bovine buttermilk
  • Solubility:ethanol, methanol, chloroform/methanol, 2:1
  • Physical Appearance:solid
  • Storage:-20°C
  • Dry Ice:No
  • Hazardous:No
  • Literature References:Application Notes:

    Glucosylsphingosine is the lyso-derivative of the common glycolipid glucocerebroside. Gaucher disease is characterized by an accumulation of glucocerebroside due to a deficiency in the enzyme glucocerebrosidase and it has now been found that glucosylsphingosine also accumulates in this disease.1 This accumulation of glucosylsphingosine contributes to neuronal dysfunction and destruction in patients with neuronopathic Gaucher disease2 and it has been found to be a potent inhibitor of glucocerebrosidase. At least some instances of Gaucher disease also have a deficiency in the activity of glucosylsphingosine beta-glucosidase, the enzyme responsible for cleaving off the glucose of glucosylsphingosine and glucocerebroside. Like glucocerebroside and galactocerebroside, glucosylsphingosine can increase Ca2 + mobilization from intracellular stores although it uses a different mechanism.3 Conduritol B epoxide (CBE), an inhibitor of beta-glucosidase, and l-phenyl-2- decanoylamino-3-morpholino-l-propanol (PDMP), an inhibitor of glucosylceramide synthase, can be used to create a model of Gaucher disease and consequently an accumulation of glucopsychosine.4

    References:
    1. E. Orvisky et al. “Glucosylsphingosine accumulation in tissues from patients with Gaucher disease: correlation with phenotype and genotype” Molecular genetics and metabolism, Vol. 76(4) pp. 262-270, 2002
    2. R. Brady et al. “Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3” Neurobiology of Disease, Vol. 14(3) pp. 595-601, 2003
    3. E. Loyd-Evans et al. “Glucosylceramide and Glucosylsphingosine Modulate Calcium Mobilization from Brain Microsomes via Different Mechanisms” The Journal of Biological Chemistry, Vol. 278 pp. 23594-23599, 2003
    4. D. Sillance et al. “Glucosylceramide modulates membrane traffic along the endocytic pathway” Journal of Lipid Research, Vol. 43 pp. 1837-1845, 2002