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Results for "

Fabry

" in MedChemExpress (MCE) Product Catalog:

11

Inhibitors & Agonists

1

Natural
Products

Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-106392
    Lucerastat

    NB-DGJ; N-(n-Butyl)deoxygalactonojirimycin

    		 Glucosylceramide Synthase (GCS) Metabolic Disease
    Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study .
    Lucerastat
  • HY-N12408
    Globotriaosylsphingosine

    lyso-Gb3

    		 Calcium Channel Cancer
    Globotriaosylsphingosine (lyso-Gb3) inhibits the growth of fibroblasts, as well as their differentiation into myofibroblasts, and collagen expression. Globotriaosylsphingosine can be used for Fabry disease research .
    Globotriaosylsphingosine
  • HY-155789
    C17 Globotriaosylceramide (d18:1/17:0)

    Ceramide trihexoside (d18:1/17:0); Gb3(d18:1)(C17:0)

    		 Others Others
    C17 Globotriaosylceramide (d18:1/17:0) is a sphygosine lipid that can be used to quantify Globotriaosylceramide in plasma and urine of patients with Fabry disease by gas chromatography-mass spectrometry (GC-MS) .
    C17 Globotriaosylceramide (d18:1/17:0)
  • HY-16743
    Ibiglustat
    2 Publications Verification

    Venglustat; SAR402671; GZ402671

    		 Glucosylceramide Synthase (GCS) Metabolic Disease
    Ibiglustat (Venglustat) is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat 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 .
    Ibiglustat
  • HY-16743B
    Ibiglustat succinate
    2 Publications Verification

    Venglustat succinate; SAR402671 succinate; GZ402671 succinate

    		 Glucosylceramide Synthase (GCS) Neurological Disease
    Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat succinate 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 .
    Ibiglustat succinate
  • HY-16743A
    Ibiglustat (L-Malic acid)
    2 Publications Verification

    Venglustat (L-Malic acid); SAR402671 (L-Malic acid); GZ402671 (L-Malic acid)

    		 Glucosylceramide Synthase (GCS) Metabolic Disease
    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 .
    Ibiglustat (L-Malic acid)
  • HY-W341625
    Trihexosylceramide (d18:1/12:0)

    		Others Inflammation/Immunology
    Trihexosylceramide (d18:1/12:0) is a type of Trihexosylceramide. In the renal tissue of heterozygous Fabry's disease, the cytoplasm of glomerular epithelial cells appears foamy. Trihexosylceramide accumulated in glomerular epithelial cells as identified by immunostaining .
    Trihexosylceramide (d18:1/12:0)
  • HY-137873
    4-Methylumbelliferyl-α-D-Glucopyranoside

    4-Methylumbelliferyl-α-D-Glucose; 4-Methylumbelliferyl-α-D-Glucoside; 4-MU-α-D-Glucopyranoside

    		 Fluorescent Dye Glycosidase Others
    4-Methylumbelliferyl-α-D-Glucopyranoside (4-Methylumbelliferyl-α-D-Glucose) is a fluorescent substrate for α-glucosidase, which releases the fluorescent moiety 4-methylumbelliferyl (4-MU) upon cleavage. 4-MU has pH-dependent fluorescence excitation activity, with excitation wavelengths of 320 nm at low pH (1.97-6.72) and 360 nm at high pH (7.12-10.3), respectively. The emission wavelength of 4-Methylumbelliferyl-α-D-Glucopyranoside increases with decreasing pH, ranging from 445-455 nm. 4-Methylumbelliferyl-α-D-Glucopyranoside can be used as a biomarker for Fabry and Pompe diseases to quantify α-glucosidase activity in infant blood spot samples.
    4-Methylumbelliferyl-α-D-Glucopyranoside
  • HY-14929A
    Migalastat hydrochloride
    1 Publications Verification

    GR181413A

    		 Others Others
    Migalastat (GR181413A free base) hydrochloride is an orally active α-galactosidase A molecular chaperone, with an IC50 value of 0.04 μM for human α-Gal A. Migalastat binds to the active site of certain unstable mutant forms of α-galactosidase A, facilitating their transport to the lysosome. After dissociation in the acidic environment, Migalastat enables the mutant α-galactosidase A to exhibit biological activity .
    Migalastat hydrochloride
  • HY-16743C
    Ibiglustat hydrochloride

    Venglustat hydrochloride; SAR402671 hydrochloride; GZ402671 hydrochloride

    		 Glucosylceramide Synthase (GCS) Metabolic Disease
    Ibiglustat hydrochloride is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat hydrochloride 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 .
    Ibiglustat hydrochloride
  • HY-14929
    Migalastat

    GR181413A free base; 1-Deoxygalactonojirimycin

    		 Others Others
    Migalastat (GR181413A free base) is an orally active α-galactosidase A molecular chaperone, with an IC50 value of 0.04 μM for human α-Gal A. Migalastat binds to the active site of certain unstable mutant forms of α-galactosidase A, facilitating their transport to the lysosome. After dissociation in the acidic environment, Migalastat enables the mutant α-galactosidase A to exhibit biological activity .
    Migalastat

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