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Fabry+disease

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By Targets:	Biochemical Assay Reagents (3) Calcium Channel (1) DNA/RNA Synthesis (1) Endogenous Metabolite (1) Glucosylceramide Synthase (GCS) (7) Glycosidase (2) Glycosyltransferase (2) Isotope-Labeled Compounds (1) Reference Standards (1)
By Research Areas:	Cancer (3) Cardiovascular Disease (2) Inflammation/Immunology (1) Metabolic Disease (10) Neurological Disease (1)

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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-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-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-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-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-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-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-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-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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BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature.

BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

MedchemExpress Validation 03

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred

MedchemExpress Validation 04

MedchemExpress Validation

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Fabry+disease

Inhibitors & Agonists

NaturalProducts

Isotope-Labeled Compounds

Natural
Products