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Sandhoff diseases

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By Targets:	Biochemical Assay Reagents (1) Glucosylceramide Synthase (GCS) (1) Glycosidase (1)
By Research Areas:	Metabolic Disease (1) Neurological Disease (1)

Inhibitors & Agonists

Biochemical Assay Reagents

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-W127461

Ganglioside GM2, Asialo Gangliotriosylceramide	Biochemical Assay Reagents	Others
Ganglioside GM2 asialo (asialo-GM2) is a glycosphingolipid containing three monosaccharide residues and one fatty acid of variable chain length, but lacks the sialic acid residue present on ganglioside M2. Asialo-GM2 is found at low or undetectable levels in normal human brains, but it accumulates in the brains of patients with Tay-Sachs disease and Sandhoff disease, which are expressed as lysosomal β- A neurodegenerative disorder characterized by hexosaminidase A and B deficiency. It also binds to various bacteria, including Pseudomonas isolated from cystic fibrosis patients. The Asialo-GM2 mixture contains ganglioside GM2 asialo molecular species with fatty acyl chains of variable length.

HY-206259

CCG-203586	Glucosylceramide Synthase (GCS)	Metabolic Disease
CCG-203586 is a brain-penetrant glucosylceramide synthase (GCS) inhibitor. CCG-203586 reduces GCS production and brain levels, blocks the first committed step in ganglioside biosynthesis, and lowers downstream ganglioside levels. CCG-203586 can be used for the research of Tay-Sachs, Sandhoff disease, and types 2 and 3 Gaucher disease .

HY-108241A

Galacto-PUGNAc	Glycosidase	Neurological Disease
Galacto-PUGNAc is a selective competitive inhibitor of lysosomal β-hexosaminidases (HEX), with K_i values of 51 and 18 nM, for hHEXA and hHEXB, respectively. Galacto-PUGNAc elevates GM2 ganglioside levels in neuroblastoma cells. Galacto-PUGNAc can be used for the research of neurodegenerative disorders .

Cat. No.	Product Name	Type

HY-W127461

Ganglioside GM2, Asialo Gangliotriosylceramide	Biochemical Assay Reagents
Ganglioside GM2 asialo (asialo-GM2) is a glycosphingolipid containing three monosaccharide residues and one fatty acid of variable chain length, but lacks the sialic acid residue present on ganglioside M2. Asialo-GM2 is found at low or undetectable levels in normal human brains, but it accumulates in the brains of patients with Tay-Sachs disease and Sandhoff disease, which are expressed as lysosomal β- A neurodegenerative disorder characterized by hexosaminidase A and B deficiency. It also binds to various bacteria, including Pseudomonas isolated from cystic fibrosis patients. The Asialo-GM2 mixture contains ganglioside GM2 asialo molecular species with fatty acyl chains of variable length.

Ganglioside GM2, Asialo

Gangliotriosylceramide

Biochemical Assay Reagents

Ganglioside GM2 asialo (asialo-GM2) is a glycosphingolipid containing three monosaccharide residues and one fatty acid of variable chain length, but lacks the sialic acid residue present on ganglioside M2. Asialo-GM2 is found at low or undetectable levels in normal human brains, but it accumulates in the brains of patients with Tay-Sachs disease and Sandhoff disease, which are expressed as lysosomal β- A neurodegenerative disorder characterized by hexosaminidase A and B deficiency. It also binds to various bacteria, including Pseudomonas isolated from cystic fibrosis patients. The Asialo-GM2 mixture contains ganglioside GM2 asialo molecular species with fatty acyl chains of variable length.

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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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