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Results for "

Fabry disease

" 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-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 Others
    4-Methylumbelliferyl-α-D-Glucopyranoside (4-Methylumbelliferyl-α-D-Glucose; 4-Methylumbelliferyl-α-D-Glucoside) is a fluorogenic substrate for α-glucosidase. 4-Methylumbelliferyl-α-D-glucopyranoside is cleaved by α-glucosidase to release the fluorescent moiety 4-methylumbelliferyl (4-MU). 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high pH (7.12-10.3), respectively, and an emission maximum ranging from 445 to 455 nm, increasing as pH decreases. 4-Methylumbelliferyl-α-D-glucopyranoside has been used to quantify α-glucosidase activity in infant blood spot samples as a biomarker of Fabry and Pompe diseases, lysosomal storage disorders characterized by a deficiency in the enzyme.
    4-Methylumbelliferyl-α-D-Glucopyranoside
  • HY-14929A
    Migalastat hydrochloride

    GR181413A

    		 Others Others
    Migalastat hydrochloride (GR181413A) is a potent and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A.
    Migalastat hydrochloride
  • 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-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

    		 Others Others
    Migalastat (GR181413A free base) is an orally active and competitive inhibitor of α-galactosidase A (α-Gal A) with an IC50 of 0.04 μM for human α-Gal A .
    Migalastat

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