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Lysosomal storage diseases

" in MedChemExpress (MCE) Product Catalog:
Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-151536
    meso-Benzothiazole-BODIPY 505/515

    		Fluorescent Dye Neurological Disease
    meso-Benzothiazole-BODIPY 505/515 is a boron dipyrromethenes (BODIPY) -based fluorescent probediseases, lysosomal storage diseases and neural degeneration diseases .
    meso-Benzothiazole-BODIPY 505/515
  • HY-151240
    Galactosylceramide

    		Endogenous Metabolite Neurological Disease
    Galactosylceramide (GalC) is a glucosylceramide that has been implicated in many physiological and pathological phenomena. For example, important lysosomal storage disorders such as Gaucher disease (GD) and neurodegenerative diseases are characterized by GlcCer accumulation.
    Galactosylceramide
  • HY-148385
    Ganglioside GM2

    		Others Cancer
    Ganglioside GM2 is a human tumor antigen (OFA-I-1). Ganglioside GM2 is the major lysosomal storage compound of Tay-Sachs disease .
    Ganglioside GM2
  • HY-147976
    Glucocerebrosidase-IN-1

    		Glucosidase Neurological Disease Metabolic Disease
    Glucocerebrosidase-IN-1 (compound 11a) is a potent and selective GCase (glucocerebrosidase) inhibitor, with an IC50 of 29.3 μM and a Ki of 18.5 μM. Glucocerebrosidase-IN-1 can be used for the research of Gaucher disease (GD) and Parkinson’s disease (PD) .
    Glucocerebrosidase-IN-1
  • HY-147976A
    Glucocerebrosidase-IN-1 hydrochloride

    		Glucosidase Neurological Disease
    Glucocerebrosidase-IN-1 (compound 11a) hydrochloride is a potent and selective GCase (glucocerebrosidase) inhibitor, with an IC50 of 29.3 μM and a Ki of 18.5 μM. Glucocerebrosidase-IN-1 hydrochloride can be used for the research of Gaucher disease (GD) and Parkinson’s disease (PD) .
    Glucocerebrosidase-IN-1 hydrochloride
  • HY-156020
    Glucocerebrosidase-IN-2

    		Glucosidase Metabolic Disease
    Glucocerebrosidase-IN-2 (compound 12) is a quinazoline analogue and an inhibitor of glucocerebrosidase (GC). Glucocerebrosidase-IN-2 has the potential to improve GC translocation to lysosomes in Gaucher disease patient-derived cells (mostly carrying the N370S mutation). Glucocerebrosidase-IN-2 inhibits the hydrolysis of 4-methylumbelliferone β-D-glucopyranoside (4MU) and fluorescent glycosylceramide (FlourGC) in N370S mutant tissues with an AC50 of 25.29 μM .
    Glucocerebrosidase-IN-2
  • HY-145549
    C20 Sphingomyelin (d18:1/20:0)

    N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine; SM(d18:1/20:0)

    		 Others Infection
    C20 Sphingomyelin is a naturally occurring sphingolipid. Levels of C20 sphingomyelin are upregulated in the hippocampus of rats with diabetes induced by streptozotocin (HY-13753) and in human plasma where it is positively correlated with insulin resistance in obese humans. C20 sphingomyelin is also upregulated in the liver of a mouse model of Niemann-Pick type C1 disease, a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder. The plasma concentration of C20 sphingomyelin is decreased in men with prostate cancer. As this product is derived from a natural source, there may be variations in the sphingoid backbone.
    C20 Sphingomyelin (d18:1/20:0)
  • HY-108721
    Velaglucerase alfa

    		Others Metabolic Disease
    Velaglucerase alfa is produced through gene activation technology and is identical to wild-type enzyme. Velaglucerase alfa targets accumulated glucocerebroside primarily within the lysosome of the macrophages in the affected organs and systems. Velaglucerase alfa is an enzyme replacement therapy (ERT) for type 1 Gaucher disease (GD) .
    Velaglucerase alfa
  • 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

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