3 Results for "

Sandhoff diseases

" in MedChemExpress (MCE) Product Catalog:
Products (3)

3 Results for "Sandhoff diseases" in MCE Product Catalog:

Cat. No.: HY-W127461
CAS No.: 35960-33-9
Synonyms: Gangliotriosylceramide
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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Cat. No.: HY-206259
CAS No.: 1430611-23-6
Research Areas:  

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 .
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Cat. No.: HY-108241A
CAS No.: 1145878-98-3
Target:  

Glycosidase

Research Areas:  

Neurological Disease

Galacto-PUGNAc is a selective competitive inhibitor of lysosomal β-hexosaminidases (HEX), with Ki 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 .
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