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Results for "

Pompe disease

" in MedChemExpress (MCE) Product Catalog:

4

Inhibitors & Agonists

1

Inhibitory Antibodies

Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-137873
    4-Methylumbelliferyl-α-D-Glucopyranoside

    4-Methylumbelliferyl-α-D-Glucose; 4-Methylumbelliferyl-α-D-Glucoside; 4-MU-α-D-Glucopyranoside

    		 Fluorescent Dye Others
    4-Methylumbelliferyl-α-D-Glucopyranoside (4-Methylumbelliferyl-α-D-Glucose; 4-Methylumbelliferyl-α-D-Glucoside) is a fluorogenic substrate for α-glucosidase. 4-Methylumbelliferyl-α-D-glucopyranoside is cleaved by α-glucosidase to release the fluorescent moiety 4-methylumbelliferyl (4-MU). 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high pH (7.12-10.3), respectively, and an emission maximum ranging from 445 to 455 nm, increasing as pH decreases. 4-Methylumbelliferyl-α-D-glucopyranoside has been used to quantify α-glucosidase activity in infant blood spot samples as a biomarker of Fabry and Pompe diseases, lysosomal storage disorders characterized by a deficiency in the enzyme.
    4-Methylumbelliferyl-α-D-Glucopyranoside
  • HY-108828
    Alglucosidase alfa

    rhGAA

    		 Others Others
    Alglucosidase alfa is a hydrolytic lysosomal glycogen-specific recombinant human α-glucosidase product. Alglucosidase alfa can be used for Pompe disease research .
    Alglucosidase alfa
  • HY-157646
    MZ-101

    GYS1-IN-2

    		 Others Metabolic Disease
    MZ-101 (GYS1-IN-2) is an orally active, potent and selective small-molecule glycogen synthase 1 (GYS1) inhibitor with with an IC50 value of 0.041 µM. MZ-101 reduces glycogen concentrations in cells and in mice. MZ-101 can used to study GYS1 -mediated Pompe disease and other glycogen storage diseases .
    MZ-101
  • HY-P99803
    Clervonafusp alfa

    VAL-1221

    		 Glucosidase Others
    Clervonafusp alfa (VAL-1221) is a fusion protein targeting both cytosolic and lysosomal glycogen. Clervonafusp alfa is comprised of the Fab portion of a cell-penetrating antibody and recombinant human acid alpha glucosidase (rhGAA), the former utilizing the nucleoside transporter ENT-2 to gain access to the cytosol, and the latter enters lysosomes via mannose-6-phosphate receptors (M6PRs). Clervonafusp alfa can be used for late-onset Pompe disease research .
    Clervonafusp alfa

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