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lysosomal storage diseases

" in MedChemExpress (MCE) Product Catalog:

17

Inhibitors & Agonists

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Natural
Products

1

Isotope-Labeled Compounds

Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-147976
    Glucocerebrosidase-IN-1

    		Glycosidase 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-141866
    Acid Ceramidase-IN-1
    1 Publications Verification

    		 Ceramidase Neurological Disease
    Acid Ceramidase-IN-1 is orally active and blood-brain barrier penetrant acid ceramidase (AC, ASAH-1) inhibitor (hAC IC50=0.166 μM). Acid Ceramidase-IN-1 reduces AC activity, accumulates ceramide species (Cer (d18:0/16:0), Cer (d18:1/16:0)), and decreases sphingosine levels. Acid Ceramidase-IN-1 can be used for the study of severe neurological lysosomal storage diseases (LSDs) such as Gaucher’s disease (GD) and Krabbe’s disease (KD) .
    Acid Ceramidase-IN-1
  • HY-153830
    LacCer (d18:1/16:0)

    C16 Lactosylceramide (d18:1/16:0)

    		 Endogenous Metabolite Neurological Disease Metabolic Disease
    LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0)) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). The levels of LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. In a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder), the expression of LacCer (d18:1/16:0) is also upregulated. LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .
    LacCer (d18:1/16:0)
  • HY-151240
    Galactosylceramide

    		Endogenous Metabolite Neurological Disease
    Galactosylceramide (GalC) is a galactosylceramide 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-W056439
    N-Boc-3-hydroxy-1,2,3,6-tetrahydropyridine

    		Glycosidase Neurological Disease
    N-Boc-3-hydroxy-1,2,3,6-tetrahydropyridine can increase the activity of β-glucocerebrosidase by 20%. N-Boc-3-hydroxy-1,2,3,6-tetrahydropyridine can be used in the study of lysosomal storage diseases and other protein storage diseases .
    N-Boc-3-hydroxy-1,2,3,6-tetrahydropyridine
  • HY-147976A
    Glucocerebrosidase-IN-1 hydrochloride

    		Glycosidase 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-145549
    C20 Sphingomyelin (d18:1/20:0)

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

    		 Endogenous Metabolite Endocrinology
    C20 Sphingomyelin (d18:1/20:0) (N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine; SM(d18:1/20:0)) is a naturally occurring sphingolipid. C20 Sphingomyelin (d18:1/20:0) levels are upregulated in the hippocampus of streptozotocin (HY-13753)-induced diabetic rats and in human plasma, positively correlating with insulin resistance in obese humans. C20 Sphingomyelin (d18:1/20:0) is also upregulated in the liver of a mouse model of Niemann-Pick C1 disease, a neurodegenerative cholesterol sphingolipid lysosomal storage disorder.
    C20 Sphingomyelin (d18:1/20:0)
  • HY-153830S
    LacCer (d18:1/16:0)-d3

    C16 Lactosylceramide (d18:1/16:0)-d3

    		 Isotope-Labeled Compounds Endogenous Metabolite Neurological Disease Metabolic Disease
    LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0))-d3 is the deuterium labeled LacCer (d18:1/16:0) (HY-153830). LacCer (d18:1/16:0) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. LacCer (d18:1/16:0) is also upregulated in a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder). LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .
    LacCer (d18:1/16:0)-d3
  • HY-178202
    5-C-phenethyl-DNJ

    		Glycosidase Metabolic Disease
    5-C-phenethyl-DNJ is a selective α-glucosidase GAA inhibitor, with its Ki value for rhGAA being 0.81 μM. 5-C-phenethyl-DNJ exhibits extremely high selectivity for GANAB, GBA1, and GBA2. 5-C-phenethyl-DNJ can be used for the study of Pompe disease .
    5-C-phenethyl-DNJ
  • 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-156020
    Glucocerebrosidase-IN-2

    		Glycosidase 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-156208
    C2 Adamantanyl galactosylceramide (d18:1/2:0)

    AdaGalCer(d18:1/2:0); Admantanyl galactosylceramide (d18:1/2:0); Admantanyl galCer(d18:1/2:0)

    		 Glycosidase Glucosylceramide Synthase (GCS) Glycosyltransferase Metabolic Disease
    C2 Adamantanyl galactosylceramide (AdaGalCer) (d18:1/2:0) is a bioactive sphingolipid. C2 Adamantanyl galactosylceramide (d18:1/2:0) stimulates glucocerebrosidase activity in vitro. C2 Adamantanyl galactosylceramide (d18:1/2:0) inhibits microsomal LacCer and Gb3 synthase, and inhibits cell sulfatide synthesis. C2 Adamantanyl galactosylceramide (d18:1/2:0) reduces glucosylceramide (GlcCer) levels in normal and lysosomal storage disease (LSD) cells. C2 Adamantanyl galactosylceramide (d18:1/2:0) acts as a substrate for A4GALT and is able to lower Gb3 levels with an IC50 concentration of 40 μM in fabry disease cells .
    C2 Adamantanyl galactosylceramide (d18:1/2:0)
  • HY-186121
    Glucosylceramide synthase-IN-5

    		Glucosylceramide Synthase (GCS) Metabolic Disease
    Glucosylceramide synthase-IN-5 (G3-3) is a glucosylceramide synthase (GCS) inhibitor that can be used for the study of the diseases and disorders associated with GCS activity, such as lysosomal storage disorders .
    Glucosylceramide synthase-IN-5
  • HY-186121A
    Glucosylceramide synthase-IN-6

    		Glucosylceramide Synthase (GCS) Metabolic Disease
    Glucosylceramide synthase-IN-6 (G3-3B) is a glucosylceramide synthase (GCS) inhibitor that can be used for the study of the diseases and disorders associated with GCS activity, such as lysosomal storage disorders .
    Glucosylceramide synthase-IN-6
  • HY-132177J
    α1-3,4 Fucosidase, Sweet almond

    		Endogenous Metabolite Metabolic Disease
    α1-3,4 Fucosidase, Sweet almond is an enzyme that breaks down fucose. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues.
    α1-3,4 Fucosidase, Sweet almond
  • HY-P2858H
    β-Mannosidase, Streptomyces coelicolor

    		Biochemical Assay Reagents Others
    β-Mannosidase, Streptomyces coelicolor (EC 3.2.1.25) catalyses the following chemical reaction:Hydrolysis of terminal, non-reducing beta-D-mannose residues in beta-D-mannosides. This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement.
    β-Mannosidase, Streptomyces coelicolor
  • 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

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