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Results for "

Tay-Sachs disease

" in MedChemExpress (MCE) Product Catalog:

5

阻害剤およびアゴニスト

1

生化学アッセイ試薬

製品番号 製品名 Target 研究分野 構造式
  • HY-148385
    Ganglioside GM2

    		Endogenous Metabolite Integrin FAK Src ERK p38 MAPK Neurological Disease Cancer
    Ganglioside GM2 is a human tumor antigen predominantly found in human tumor cells and fetal brain tissue. As a sialylated glycosphingolipid, Ganglioside GM2 is involved in processes such as cell signaling, adhesion, and motility. Ganglioside GM2 abnormal expression and accumulation are associated with tumors and neurodegenerative disorders. Ganglioside GM2 promotes tumor cell migration and invasion by directly binding to the integrin β1 receptor, activating the FAK/Src/Erk-MAPK signaling pathway, and inducing actin cytoskeleton remodeling .
    Ganglioside GM2
  • 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 .
    Lyso-monosialoganglioside GM2 ammonium
  • 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.
    Ganglioside GM2, Asialo
  • 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 .
    CCG-203586
  • HY-108241A
    Galacto-PUGNAc

    		Glycosidase 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 .
    Galacto-PUGNAc

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