lysosomal disorders

By Targets:	Biochemical Assay Reagents (1) Fluorescent Dye (1) Glucosylceramide Synthase (GCS) (1) UGT (1)
By Research Areas:	Metabolic Disease (1) Neurological Disease (1)

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-148786

Nizubaglustat	Glucosylceramide Synthase (GCS)	Neurological Disease
Nizubaglustat is an inhibitor of Ceramide Glucosyltransferase or Glucosylceramide Synthase (GCS). Nizubaglustat also inhibits GBA2, involving in neuropathological lysosomal storage disorders .

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.

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.

MCE Japan Authorized Agent ナミキ商事株式会社コスモ・バイオ株式会社
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Inhibitors & Agonists

Fluorescent Dye

Biochemical Assay Reagents