lysosomal disorders

By Targets:	Biochemical Assay Reagents (1) Endogenous Metabolite (2) Fluorescent Dye (2) Glucosidase (1) UGT (1)
By Research Areas:	Infection (1) Metabolic Disease (2) Neurological Disease (2)

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-131703

UGT8-IN-1	UGT	Metabolic Disease
UGT8-IN-1 is a brain penetrable and orally active inhibitor of ceramide galactosyltransferase enzyme (UGT8). UGT8-IN-1 can be used in the study for lysosomal storage disorders .

HY-D1632

4-MU-α-GlcNS sodium	Fluorescent Dye	Others
4-MU-α-GlcNS sodium is a fluorogenic substrate of heparin sulphamidase, is desulfurized into 4-MU-α-GlcNH₂. 4-MU-α-GlcNH₂ can liberate 4-methylumbelliferone (4-MU, fluorescent product) via α-glucosaminidase catalysis, with the emission wavelength maxima of 445-454 nm. 4-MU-α-GlcNS sodium can be used to heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders researches .

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.

HY-E70305

GlcNAc-1-Phosphotransferase	Endogenous Metabolite	Neurological Disease
GlcNAc-1-Phosphotransferase is an α2β2γ2 hexamer with core catalytic α- and β-subunits derived from GNPTAB. GlcNAc-1-Phosphotransferase catalyzes the addition of the il group of N-acetylglucosamine-1-phosphate to the terminal mannose. Mutations in GlcNAc-1-Phosphotransferase cause lysosomal storage disorders such as mucolipidosis .

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 .

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.

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.

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.

Cat. No.	Product Name	Type

HY-D1632

4-MU-α-GlcNS sodium	Fluorescent Dyes/Probes
4-MU-α-GlcNS sodium is a fluorogenic substrate of heparin sulphamidase, is desulfurized into 4-MU-α-GlcNH₂. 4-MU-α-GlcNH₂ can liberate 4-methylumbelliferone (4-MU, fluorescent product) via α-glucosaminidase catalysis, with the emission wavelength maxima of 445-454 nm. 4-MU-α-GlcNS sodium can be used to heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders researches .

4-MU-α-GlcNS sodium

Fluorescent Dyes/Probes

4-MU-α-GlcNS sodium is a fluorogenic substrate of heparin sulphamidase, is desulfurized into 4-MU-α-GlcNH₂. 4-MU-α-GlcNH₂ can liberate 4-methylumbelliferone (4-MU, fluorescent product) via α-glucosaminidase catalysis, with the emission wavelength maxima of 445-454 nm. 4-MU-α-GlcNS sodium can be used to heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders researches .

Cat. No.	Product Name	Type

HY-W127461

Ganglioside GM2, Asialo Gangliotriosylceramide	Biochemical Assay Reagents
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

Gangliotriosylceramide

Biochemical Assay Reagents

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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Inhibitors & Agonists

Fluorescent Dye

Biochemical Assay Reagents