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Lysosomal Storage Disease

Lysosomal storage diseases (LSDs) are a group of rare, inherited metabolic disorders characterized by the deficiency of specific lysosomal enzymes. This deficiency leads to the abnormal accumulation of undigested or partially digested macromolecules within lysosomes, causing cellular dysfunction and progressive damage to various organs and tissues. LSDs present with a wide spectrum of clinical manifestations affecting neurological, skeletal, visceral, and hematological systems, often leading to severe and life-limiting conditions.
Catalog No Product Description CAS No. Purity Structural Formula
BP0030
1-Deoxynojirimycin
1-Deoxynojirimycin is an optically active form of 2-(hydroxymethyl)piperidine-3,4,5-triol having 2R,3R,4R,5S-configuration. It has a role as a hypoglycemic agent, a hepatoprotective agent, an anti-HIV agent, an EC 3.2.1.20 (alpha-glucosidase) inhibitor, an anti-obesity agent, a plant metabolite and a bacterial metabolite. It is a piperidine alkaloid and a 2-(hydroxymethyl)piperidine-3,4,5-triol. 1-Deoxynojirimycin is a potent α-glucosidase inhibitor, suppresses postprandial blood glucose, thereby possibly preventing diabetes mellitus. 1-Deoxynojirimycin as a therapeutic agent by controlling the overgrowth and biofilm formation of S. mutans, it also can block human immunodeficiency virus envelope glycoprotein- mediated membrane fusion at the CXCR4 binding step. 1-Deoxynojirimycin is an orally effective alpha glucosidase inhibitor. 1-Deoxynojirimycin inhibits postprandial blood glucose and prevents diabetes. 1-Deoxynojirimycin has hypoglycemic, weight loss, and antiviral effects.
19130-96-2 99% 1-Deoxynojirimycin
BP5229
Miglustat
Miglustat is a hydroxypiperidine that is deoxynojirimycin in which the amino hydrogen is replaced by a butyl group. It has a role as an EC 2.4.1.80 (ceramide glucosyltransferase) inhibitor and an anti-HIV agent. It is a member of piperidines and a tertiary amino compound. It is functionally related to a duvoglustat.
72599-27-0 98% Miglustat
BP4546
Diphyllin
Diphyllin could be characterized as a new V-ATPase inhibitor in treating gastric cancer and inhibiting the phosphorylation of LRP6 in Wnt/β-catenin signaling. Diphyllin has anti-inflammatory activities, it shows 50% inhibition of NO production from peritoneal macrophages. Diphyllin also has in vitro antileishmanial activity, it exerts a strong specific inhibitory activity (IC50 = 0.2 microM) resulting from the inhibition of parasite internalization within macrophages.
22055-22-7 98% Diphyllin