| Size | Price | Stock | Qty |
|---|---|---|---|
| 1mg |
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| 5mg |
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| Other Sizes |
| Targets |
Glucocerebrosidase (GBA). It is also a substrate for the enzyme and a biomarker for Gaucher disease.
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|---|---|
| ln Vitro |
Glucosylsphingosine is a non-competitive inhibitor of glucocerebrosidase with a Ki of 30 μM. It is a neurotoxic compound and plays a role in inflammation, immune activation, and neuronal dysfunction. Its accumulation is a hallmark of Gaucher disease.
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| ln Vivo |
In vivo, glucosylsphingosine is a critical biomarker for Gaucher disease. Elevated levels are found in the plasma and tissues of patients with this lysosomal storage disorder. Its concentration correlates with disease severity and is used to monitor the efficacy of enzyme replacement therapy.
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| Enzyme Assay |
The inhibition of glucocerebrosidase by glucosylsphingosine can be measured using an enzyme activity assay. The enzyme is incubated with its substrate and varying concentrations of the inhibitor. The remaining enzyme activity is measured, and the Ki value (30 μM) is determined from the kinetic data.
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| Cell Assay |
Glucosylsphingosine is not typically used as a treatment in cell culture, but its levels are measured in cells from patients or in cellular models of Gaucher disease. Its accumulation can be quantified by LC-MS/MS to assess disease status or the efficacy of therapeutic interventions.
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| Animal Protocol |
Animal models of Gaucher disease are used to study the pathophysiology and to evaluate potential therapies. The levels of glucosylsphingosine in plasma and tissues are measured as a key pharmacodynamic marker to assess the efficacy of treatments designed to reduce its accumulation.
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| ADME/Pharmacokinetics |
As a lipid metabolite, glucosylsphingosine is not a drug with conventional PK properties. Its concentration in plasma and tissues is a biomarker. Its levels are tightly regulated by the balance between its synthesis (from glucosylceramide) and its degradation (by glucocerebrosidase).
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| Toxicity/Toxicokinetics |
Glucosylsphingosine itself is not considered a toxic compound at normal levels, but its accumulation in Gaucher disease is associated with toxicity and pathology, contributing to the clinical manifestations of the disease. Its neurotoxicity is a key feature of the disease.
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| References | |
| Additional Infomation |
β-D-glucosylsphingosine is a D-glucosylsphingosine with a β-meric anomeric configuration at the glucose syl group. It is a D-glucosylsphingosine and also a β-D-glucoside. It is the conjugate base of β-D-glucosylsphingosine (1+).
Glucosylsphingosine is a critical biomarker for the diagnosis and monitoring of Gaucher disease. Its measurement in dried blood spots has become a standard newborn screening method and a tool for monitoring disease progression and treatment response in patients. |
| Molecular Formula |
C24H47NO7
|
|---|---|
| Molecular Weight |
461.63
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| Exact Mass |
461.335
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| CAS # |
52050-17-6
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| Related CAS # |
Glucosylsphingosine-d7;2315262-29-2;Glucosylsphingosine-d5;Glucosylsphingosine-13C6;299172-48-8
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| PubChem CID |
5280570
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| Appearance |
White to off-white solid powder
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| Density |
1.14g/cm3
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| Boiling Point |
664.3ºC at 760mmHg
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| Flash Point |
355.6ºC
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| Index of Refraction |
1.535
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| LogP |
2.448
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| Hydrogen Bond Donor Count |
6
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| Hydrogen Bond Acceptor Count |
8
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| Rotatable Bond Count |
18
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| Heavy Atom Count |
32
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| Complexity |
479
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| Defined Atom Stereocenter Count |
7
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| SMILES |
CCCCCCCCCCCCC/C=C/[C@H]([C@H](CO[C@H]1[C@@H]([C@H]([C@@H]([C@H](O1)CO)O)O)O)N)O
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| InChi Key |
HHJTWTPUPVQKNA-JIAPQYILSA-N
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| InChi Code |
InChI=1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18-,19+,20+,21+,22-,23+,24+/m0/s1
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| Chemical Name |
(2R,3R,4S,5S,6R)-2-[(E,2S,3R)-2-amino-3-hydroxyoctadec-4-enoxy]-6-(hydroxymethyl)oxane-3,4,5-triol
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| HS Tariff Code |
2934.99.9001
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| Storage |
Powder -20°C 3 years 4°C 2 years In solvent -80°C 6 months -20°C 1 month Note: This product requires protection from light (avoid light exposure) during transportation and storage. |
| Shipping Condition |
Room temperature (This product is stable at ambient temperature for a few days during ordinary shipping and time spent in Customs)
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| Solubility (In Vitro) |
H2O: 125 mg/mL (270.78 mM)
DMSO: 100 mg/mL (216.62 mM) |
|---|---|
| Solubility (In Vivo) |
Solubility in Formulation 1: ≥ 2.5 mg/mL (5.42 mM) (saturation unknown) in 10% DMSO + 40% PEG300 + 5% Tween80 + 45% Saline (add these co-solvents sequentially from left to right, and one by one), clear solution.
For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 25.0 mg/mL clear DMSO stock solution to 400 μL PEG300 and mix evenly; then add 50 μL Tween-80 to the above solution and mix evenly; then add 450 μL normal saline to adjust the volume to 1 mL. Preparation of saline: Dissolve 0.9 g of sodium chloride in 100 mL ddH₂ O to obtain a clear solution. Solubility in Formulation 2: ≥ 2.5 mg/mL (5.42 mM) (saturation unknown) in 10% DMSO + 90% (20% SBE-β-CD in Saline) (add these co-solvents sequentially from left to right, and one by one), clear solution. For example, if 1 mL of working solution is to be prepared, you can add 100 μL of 25.0 mg/mL clear DMSO stock solution to 900 μL of 20% SBE-β-CD physiological saline solution and mix evenly. Preparation of 20% SBE-β-CD in Saline (4°C，1 week): Dissolve 2 g SBE-β-CD in 10 mL saline to obtain a clear solution. View More
Solubility in Formulation 3: ≥ 2.5 mg/mL (5.42 mM) (saturation unknown) in 10% DMSO + 90% Corn Oil (add these co-solvents sequentially from left to right, and one by one), clear solution. |
| Preparing Stock Solutions | 1 mg | 5 mg | 10 mg | |
| 1 mM | 2.1662 mL | 10.8312 mL | 21.6624 mL | |
| 5 mM | 0.4332 mL | 2.1662 mL | 4.3325 mL | |
| 10 mM | 0.2166 mL | 1.0831 mL | 2.1662 mL |
*Note: Please select an appropriate solvent for the preparation of stock solution based on your experiment needs. For most products, DMSO can be used for preparing stock solutions (e.g. 5 mM, 10 mM, or 20 mM concentration); some products with high aqueous solubility may be dissolved in water directly. Solubility information is available at the above Solubility Data section. Once the stock solution is prepared, aliquot it to routine usage volumes and store at -20°C or -80°C. Avoid repeated freeze and thaw cycles.
Calculation results
Working concentration： mg/mL；
Method for preparing DMSO stock solution： mg drug pre-dissolved in μL DMSO (stock solution concentration mg/mL). Please contact us first if the concentration exceeds the DMSO solubility of the batch of drug.
Method for preparing in vivo formulation:：Take μL DMSO stock solution, next add μL PEG300, mix and clarify, next addμL Tween 80, mix and clarify, next add μL ddH2O，mix and clarify.
(1) Please be sure that the solution is clear before the addition of next solvent. Dissolution methods like vortex, ultrasound or warming and heat may be used to aid dissolving.
(2) Be sure to add the solvent(s) in order.
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