2. Neuronal Signaling
  3. Glucosylceramide Synthase (GCS)
  4. Ibiglustat (L-Malic acid)

Ibiglustat (L-Malic acid)  (Synonyms: Venglustat (L-Malic acid); SAR402671 (L-Malic acid); GZ402671 (L-Malic acid))

Cat. No.: HY-16743A Purity: 98.65%
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Ibiglustat (Venglustat) L-Malic acid is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat L-Malic acid can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease.

For research use only. We do not sell to patients.

CAS No. : 1629063-78-0

Size Price Stock Quantity
Solid + Solvent (Highly Recommended)
10 mM * 1 mL in DMSO
ready for reconstitution
 In-stock
Solution
10 mM * 1 mL in DMSO In-stock
Solid
2 mg In-stock
5 mg In-stock
10 mg In-stock
50 mg   Get quote  
100 mg   Get quote  

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This product is a controlled substance and not for sale in your territory.

Customer Review

Based on 3 publication(s) in Google Scholar

Other Forms of Ibiglustat (L-Malic acid):

Ibiglustat (L-Malic acid) Related Antibodies

Top Publications Citing Use of Products
Cell Proliferation/Viability Assay
Flow Cytometry
WB
IF
Histological Imaging/Staining

    Ibiglustat (L-Malic acid) purchased from MedChemExpress. Usage Cited in: Int J Biol Macromol. 2024 Dec 2:285:138303.  [Abstract]

    CCK-8 assay evaluating the proliferation of PK-15 cells treated with UGCG inhibitors Ibiglustat (1.6-1000 nM) over 12-36 h.

    Ibiglustat (L-Malic acid) purchased from MedChemExpress. Usage Cited in: Int J Biol Macromol. 2024 Dec 2:285:138303.  [Abstract]

    Flow cytometry analysis of PRV-GFP infection in PK-15 cells pretreated with UGCG inhibitors Eliglustat hemitartrate (1.6–1000 nM) and Ibiglustat (1.6–1000 nM) for 3 h, followed by GFP fluorescence intensity measurement 24 h post-infection to assess viral replication. The results showed that Ibiglustat significantly reduced PRV-GFP fluorescence signals in a dose-dependent manner.

    Ibiglustat (L-Malic acid) purchased from MedChemExpress. Usage Cited in: Int J Biol Macromol. 2024 Dec 2:285:138303.  [Abstract]

    Ibiglustat (1.6–1000 nM; pretreated 3 h) reduced PRV-gB protein levels in a dose-dependent manner in PRV-QXX-infected PK-15 cells.

    Ibiglustat (L-Malic acid) purchased from MedChemExpress. Usage Cited in: Int J Biol Macromol. 2024 Dec 2:285:138303.  [Abstract]

    Ibiglustat (1.6–1000 nM; 3 h) reduced PRV-gB protein levels in a dose-dependent manner in PRV-QXX-infected PK-15 cells.

    Ibiglustat (L-Malic acid) purchased from MedChemExpress. Usage Cited in: Int J Biol Macromol. 2024 Dec 2:285:138303.  [Abstract]

    Brain, lung, liver, and spleen injury were assessed using hematoxylin and eosin staining 3 days post-infection (n = 3 per group). Histopathological examination revealed that PRV infection in mice induced proliferation of brain microglial cells, inflammatory cell infiltration in the lungs, significant thickening of alveolar septa, vacuolar degeneration of liver cells, and inflammatory tissue damage in the spleen. These pathological changes were attenuated by treatment with the UGCG inhibitors, Eliglustat hemitartrate or Ibiglustat ((2.5 mg/kg); s.c.; once daily for the first 3 days of PRV infection). Scale bar = 50 μm.

    • Biological Activity

    • Purity & Documentation

    • References

    • Customer Review

    Description

    Ibiglustat (Venglustat) L-Malic acid is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat L-Malic acid can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease[1][2].

    IC50 & Target

    Glucosylceramide synthase[1].
    In Vitro

    Ibiglustat (SAR402671) (1 μM, 15 days) L-Malic acid treated Fabry disease (FD) cells are close to the physiological level in untreated WT cells in GL-3 levels, suggesting that Ibiglustat L-Malic acid can prevent additional GL-3 accumulation and could serve to ameliorate the abundant levels of this sphingolipid in FD cardiomyocytes[4].

    MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

    Ibiglustat (L-Malic acid) Related Antibodies
    Molecular Weight

    523.57

    Formula

    C24H30FN3O7S
    CAS No.
    Appearance

    Solid

    Color

    White to off-white

    SMILES

    O=C(O[C@@H]1CN2CCC1CC2)NC(C)(C3=CSC(C4=CC=C(F)C=C4)=N3)C.OC(C[C@H](O)C(O)=O)=O
    Shipping

    Room temperature in continental US; may vary elsewhere.
    Storage
    Powder -20°C 3 years
    4°C 2 years
    In solvent -80°C 6 months
    -20°C 1 month
    Solvent & Solubility
    In Vitro: 

    DMSO : ≥ 100 mg/mL (191.00 mM; Hygroscopic DMSO has a significant impact on the solubility of product, please use newly opened DMSO)

    *"≥" means soluble, but saturation unknown.

    Preparing
    Stock Solutions
    Concentration Solvent Mass 1 mg 5 mg 10 mg
    1 mM 1.9100 mL 9.5498 mL 19.0996 mL
    5 mM 0.3820 mL 1.9100 mL 3.8199 mL
    View the Complete Stock Solution Preparation Table

    * Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
    Storage method and period of stock solution: -80°C, 6 months; -20°C, 1 month. When stored at -80°C, please use it within 6 months. When stored at -20°C, please use it within 1 month.

    • Molarity Calculator

    • Dilution Calculator

    Mass (g) = Concentration (mol/L) × Volume (L) × Molecular Weight (g/mol)

    Mass
    =
    Concentration
    ×
    Volume
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    Molecular Weight *

    Concentration (start) × Volume (start) = Concentration (final) × Volume (final)

    This equation is commonly abbreviated as: C1V1 = C2V2

    Concentration (start)

    C1

    ×
    Volume (start)

    V1

    =
    Concentration (final)

    C2

    ×
    Volume (final)

    V2

    In Vivo:

    Select the appropriate dissolution method based on your experimental animal and administration route.

    For the following dissolution methods, please ensure to first prepare a clear stock solution using an In Vitro approach and then sequentially add co-solvents:
    To ensure reliable experimental results, the clarified stock solution can be appropriately stored based on storage conditions. As for the working solution for in vivo experiments, it is recommended to prepare freshly and use it on the same day.
    The percentages shown for the solvents indicate their volumetric ratio in the final prepared solution. If precipitation or phase separation occurs during preparation, heat and/or sonication can be used to aid dissolution.

    • Protocol 1

      Add each solvent one by one:  10% DMSO    40% PEG300    5% Tween-80    45% Saline

      Solubility: ≥ 2.5 mg/mL (4.77 mM); Clear solution

      This protocol yields a clear solution of ≥ 2.5 mg/mL (saturation unknown).

      Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (25.0 mg/mL) to 400 μL PEG300, and mix evenly; then add 50 μL Tween-80 and mix evenly; then add 450 μL Saline to adjust the volume to 1 mL.

      Preparation of Saline: Dissolve 0.9 g sodium chloride in ddH₂O and dilute to 100 mL to obtain a clear Saline solution.
    • Protocol 2

      Add each solvent one by one:  10% DMSO    90% (20% SBE-β-CD in Saline)

      Solubility: ≥ 2.5 mg/mL (4.77 mM); Clear solution

      This protocol yields a clear solution of ≥ 2.5 mg/mL (saturation unknown).

      Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (25.0 mg/mL) to 900 μL 20% SBE-β-CD in Saline, and mix evenly.

      Preparation of 20% SBE-β-CD in Saline (4°C, storage for one week): 2 g SBE-β-CD powder is dissolved in 10 mL Saline, completely dissolve until clear.
    In Vivo Dissolution Calculator
    Please enter the basic information of animal experiments:

    Dosage

    mg/kg

    Animal weight
    (per animal)

    g

    Dosing volume
    (per animal)

    μL

    Number of animals

    Recommended: Prepare an additional quantity of animals to account for potential losses during experiments.
    Please enter your animal formula composition:
    %
    DMSO +
    % 
    +
    %
    Tween-80 +
    %
    Saline
    Recommended: Keep the proportion of DMSO in working solution below 2% if your animal is weak.
    The co-solvents required include: DMSO, . All of co-solvents are available by MedChemExpress (MCE). , Tween 80. All of co-solvents are available by MedChemExpress (MCE).
    Calculation results:
    Working solution concentration: mg/mL
    Method for preparing stock solution: mg drug dissolved in μL  DMSO (Stock solution concentration: mg/mL).
    The concentration of the stock solution you require exceeds the measured solubility. The following solution is for reference only. If necessary, please contact MedChemExpress (MCE).
    Method for preparing in vivo working solution for animal experiments: Take μL DMSO stock solution, add μL . μL , mix evenly, next add μL Tween 80, mix evenly, then add μL Saline.
     If the continuous dosing period exceeds half a month, please choose this protocol carefully.
    Please ensure that the stock solution in the first step is dissolved to a clear state, and add co-solvents in sequence. You can use ultrasonic heating (ultrasonic cleaner, recommended frequency 20-40 kHz), vortexing, etc. to assist dissolution.
    Purity & Documentation
    References

    Complete Stock Solution Preparation Table

    * Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
    Storage method and period of stock solution: -80°C, 6 months; -20°C, 1 month. When stored at -80°C, please use it within 6 months. When stored at -20°C, please use it within 1 month.

    Optional Solvent Concentration Solvent Mass 1 mg 5 mg 10 mg 25 mg
    DMSO 1 mM 1.9100 mL 9.5498 mL 19.0996 mL 47.7491 mL
    5 mM 0.3820 mL 1.9100 mL 3.8199 mL 9.5498 mL
    10 mM 0.1910 mL 0.9550 mL 1.9100 mL 4.7749 mL
    15 mM 0.1273 mL 0.6367 mL 1.2733 mL 3.1833 mL
    20 mM 0.0955 mL 0.4775 mL 0.9550 mL 2.3875 mL
    25 mM 0.0764 mL 0.3820 mL 0.7640 mL 1.9100 mL
    30 mM 0.0637 mL 0.3183 mL 0.6367 mL 1.5916 mL
    40 mM 0.0477 mL 0.2387 mL 0.4775 mL 1.1937 mL
    50 mM 0.0382 mL 0.1910 mL 0.3820 mL 0.9550 mL
    60 mM 0.0318 mL 0.1592 mL 0.3183 mL 0.7958 mL
    80 mM 0.0239 mL 0.1194 mL 0.2387 mL 0.5969 mL
    100 mM 0.0191 mL 0.0955 mL 0.1910 mL 0.4775 mL
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    Ibiglustat (L-Malic acid) Related Classifications

    Help & FAQs
    • Do most proteins show cross-species activity?

      Species cross-reactivity must be investigated individually for each product. Many human cytokines will produce a nice response in mouse cell lines, and many mouse proteins will show activity on human cells. Other proteins may have a lower specific activity when used in the opposite species.

    Keywords:

    Ibiglustat (L-Malic acid)1629063-78-0Venglustat (L-Malic acid)SAR402671 (L-Malic acid)GZ402671 (L-Malic acid)Glucosylceramide Synthase (GCS)GBAmutationsoralGL-3 levelsFabry diseaseParkinson's diseaseInhibitorinhibitorinhibit

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