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Fabry disease

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Biochemical Assay Reagents (3) Calcium Channel (1) DNA/RNA Synthesis (1) Endogenous Metabolite (1) Fluorescent Dye (1) Glucosylceramide Synthase (GCS) (7) Glycosidase (5) Glycosyltransferase (2) Isotope-Labeled Compounds (1) PKC (1) Reference Standards (1)
By Research Areas:	Cancer (3) Cardiovascular Disease (2) Inflammation/Immunology (1) Metabolic Disease (11) Neurological Disease (1)

Inhibitors & Agonists

Natural
Products

Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-16743

Ibiglustat 3 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 .

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 .

HY-14929A

Migalastat hydrochloride 1 Publications Verification GR181413A	Glycosidase	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 .

HY-N12408

Globotriaosylsphingosine Lyso-Gb3; Lyso-GL3	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 .

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.

HY-16743A

Ibiglustat (L-Malic acid) 3 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 .

HY-16743B

Ibiglustat succinate 3 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 .

HY-156209

Gb3(d18:1/16:0)	Biochemical Assay Reagents	Cardiovascular Disease Cancer
Gb3 (d18:1/16:0) (Compound 1) is the P ^k antigen of the P blood group system. Gb3 (d18:1/16:0) acts as a cell surface receptor. Gb3 (d18:1/16:0) is used for research on Burkitt's lymphoma, teratocarcinoma, embryonal carcinoma, and Fabry's disease .

HY-156155

GYS1-IN-1	Glycosyltransferase	Metabolic Disease
GYS1-IN-1 (compound 783) is a glycogen synthase 1(GYS1) inhibitor. GYS1-IN-1 can be used for the study of GYS1 -mediated disease, such as Gaucher and Fabry diseases .

HY-172552

RECTAS-2.0	DNA/RNA Synthesis	Metabolic Disease
RECTAS-2.0 is a small molecule designed to correct RNA mis-splicing caused by the GLA c.639+919G>A mutation, used for the research of Fabry disease .

HY-14929

Migalastat GR181413A free base; 1-Deoxygalactonojirimycin	Glycosidase	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 .

HY-172546

Lyso-monosialoganglioside GM2 ammonium LysoGM2 ammonium	PKC	Metabolic Disease
Lyso-monosialoganglioside GM2 (LysoGM2) ammonium is a lysosphingolipid and protein kinase C inhibitor (IC₅₀: 50 μM). Lyso-monosialoganglioside GM2 ammonium can inhibit the binding of protein kinase C to Phorbol 12,13-dibutyrate (HY-18985). Lyso-monosialoganglioside GM2 ammonium is a specific biomarker for GM2 gangliosidosis. Lyso-monosialoganglioside GM2 ammonium can be used in the research of sphingolipidoses .

HY-156209S

Gb3(d18:1/16:0)-d9	Isotope-Labeled Compounds Biochemical Assay Reagents	Cardiovascular Disease Cancer
Gb3(d₁₈:1/16:0)-d₉ is deuterium labeled Gb3(d18:1/16:0) (HY-156209). Gb3 (d18:1/16:0) (Compound 1) is the P ^k antigen of the P blood group system. Gb3 (d18:1/16:0) acts as a cell surface receptor. Gb3 (d18:1/16:0) is used for research on Burkitt's lymphoma, teratocarcinoma, embryonal carcinoma, and Fabry's disease .

HY-106392R

Lucerastat (Standard) NB-DGJ (Standard); N-(n-Butyl)deoxygalactonojirimycin (Standard)	Reference Standards Glucosylceramide Synthase (GCS)	Metabolic Disease
Lucerastat (Standard) is the analytical standard of Lucerastat. This product is intended for research and analytical applications. Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study .

HY-155789

C17 Globotriaosylceramide (d18:1/17:0) Ceramide trihexoside (d18:1/17:0); Gb3(d18:1)(C17:0)	Biochemical Assay Reagents	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) .

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 .

HY-156208

C2 Adamantanyl galactosylceramide (d18:1/2:0) AdaGalCer(d18:1/2:0); Admantanyl galactosylceramide (d18:1/2:0); Admantanyl galCer(d18:1/2:0)	Glycosidase Glucosylceramide Synthase (GCS) Glycosyltransferase	Metabolic Disease
C2 Adamantanyl galactosylceramide (AdaGalCer) (d18:1/2:0) is a bioactive sphingolipid. C2 Adamantanyl galactosylceramide (d18:1/2:0) stimulates glucocerebrosidase activity in vitro. C2 Adamantanyl galactosylceramide (d18:1/2:0) inhibits microsomal LacCer and Gb₃ synthase, and inhibits cell sulfatide synthesis. C2 Adamantanyl galactosylceramide (d18:1/2:0) reduces glucosylceramide (GlcCer) levels in normal and lysosomal storage disease (LSD) cells. C2 Adamantanyl galactosylceramide (d18:1/2:0) acts as a substrate for A4GALT and is able to lower Gb₃ levels with an IC₅₀ concentration of 40 μM in fabry disease cells .

HY-181469

C2 Adamantanyl glucosylceramide (d18:1/2:0) Adamantanyl GluCer (d18:1/2:0); Adamantanyl glucosylceramide (d18:1/2:0); AdaGluCer (d18:1/2:0)	Glycosidase	Metabolic Disease
C2 Adamantanyl glucosylceramide (d18:1/2:0) (Adamantanyl GluCer (d18:1/2:0)) is an inhibitor of glucocerebrosidase (GCC) and LacCer synthase. At low doses, C2 Adamantanyl glucosylceramide (d18:1/2:0) increases intracellular glycolipid levels by inhibiting glucocerebrosidase. C2 Adamantanyl glucosylceramide (d18:1/2:0) alters glycolipid metabolism. C2 Adamantanyl glucosylceramide (d18:1/2:0) can be used for the research of Gaucher disease and Fabry disease .

HY-175076

Globotriaosylceramide (non-hydroxy) (porcine RBC)	Endogenous Metabolite	Metabolic Disease Inflammation/Immunology
Globotriaosylceramide (non-hydroxy) (porcine RBC) is a glycosphingolipid found in the cell membranes of mammals. Globotriaosylceramide (non-hydroxy) (porcine RBC) is the core pathogenic molecule of Fabry disease, and its level can be downregulated by supplementing α-galactosidase A. Globotriaosylceramide (non-hydroxy) (porcine RBC) amplifies B-cell receptor signals by regulating the localization of co-receptor CD19, and can be used as a vaccine adjuvant, capable of guiding the generation of broad-spectrum and cross-protective antibody responses. Globotriaosylceramide (non-hydroxy) (porcine RBC) promotes wound healing and reduces scar formation by regulating fibroblast heterogeneity .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-N12408

Globotriaosylsphingosine Lyso-Gb3; Lyso-GL3	Structural Classification Animals Lipid Source Classification	Calcium Channel
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 .

Cat. No.	Product Name	Chemical Structure

HY-156209S

Gb3(d18:1/16:0)-d9
Gb3(d₁₈:1/16:0)-d₉ is deuterium labeled Gb3(d18:1/16:0) (HY-156209). Gb3 (d18:1/16:0) (Compound 1) is the P ^k antigen of the P blood group system. Gb3 (d18:1/16:0) acts as a cell surface receptor. Gb3 (d18:1/16:0) is used for research on Burkitt's lymphoma, teratocarcinoma, embryonal carcinoma, and Fabry's disease .

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Fabry disease

Inhibitors & Agonists

NaturalProducts

Isotope-Labeled Compounds

Natural
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