gcase

Gcase activator 1 (LTI-291) is a glucocerebrosidase (Gcase) activator.

Ambroxol (NA-872) is a secretolytic agent used in the treatment of respiratory diseases associated with viscid or excessive mucus. It is the active ingredient of Mucosolvan, Lasolvan or Mucoangin. The substance is a mucoactive drug with several properties including secretolytic and secretomotoric actions that restore the physiological clearance mechanisms of the respiratory tract which play an important role in the body's natural defense mechanisms. It stimulates synthesis and release of surfactant by type II pneumocytes. Surfactants act as an anti-glue factor by reducing the adhesion of mucus to the bronchial wall, in improving its transport and in providing protection against infection and irritating agents.

Ambroxol hydrochloride (Mucosolvan) is a metabolite of BROMHEXINE that stimulates mucociliary action and clears the air passages in the respiratory tract. It is usually administered as the hydrochloride.

Afegostat is a pharmacological chaperone that specifically and reversibly binds acid-β-glucosidase (GCase) in the endoplasmic reticulum with high affinity.

Afegostat D-Tartrate is a pharmacological chaperone that binds specifically and reversibly with high affinity to acid-β-glucosidase (GCase) in the endoplasmic reticulum.

Glucocerebrosidase (Glucosylceramidase; GBA), a lysosomal enzyme, catalyzes the hydrolysis of the β-glucosidic linkage in glucocerebroside (GC) to yield glucose and ceramide [1].

ML198 is a novel activator and non-inhibitory chaperone of glucocerebrosidase. ML198 serves as a starting point to develop a novel approach towards small molecule treatment for patients suffering from Gaucher disease.

Gcase activator 2 is a β-glucocerebrosidase activator that induces dimerization of GCase at the endoplasmic reticulum (ER) and lysosomal pHs, increases lysosomal substrate metabolism, and has therapeutic potential for the treatment of Parkinson's and Gosheimer's diseases.

GCase Modulator-1 (Compound 9g), a quinazoline derivative, functions as a glucosidase modulator with an AC50 value of 2.23 µM [1].

Gcase activator 3 is a glucocerebrosidase (GCase) activator, promoting increased GCase activity in GBA1 mutant fibroblasts. Gcase activator 3 is useful for studying Parkinson's disease.

Endoglycoceramidase II (EGCase II), an endo-β-glucosidase, catalyzes the hydrolysis of the β-glycosidic linkage between oligosaccharides and ceramides in glycosphingolipids, releasing the complete glycan from ceramide [1] [2].

Endoglycoceramidase I (EGCase I), a glycosidase frequently utilized in biochemical studies, catalyzes a transglycosylation reaction. This process involves transferring the sugar moiety of GSLs to the primary hydroxyl group of various 1-alkanols [1].

Recombinant endoglycoceramidase II (rEGCase II) is an endo-β-glucosidase that releases intact glycans from the ceramide portion of sphingolipids. It catalyzes the hydrolysis of the β-glycosidic bond between oligosaccharides and ceramide in various sphingolipids.

Recombinant endoglycoceramidase I assisted by activator II (rEGCase I assisted by Activator II) is a mixture of EGCase I and Activator II. EGCase I is a specific hydrolase ideal for comprehensive analysis of glycolipid compounds (GSLs). Activator II enhances the activity of EGCase without requiring any detergents.

Recombinant endoglycoceramidase I (rEGCase I) is a glycosidase that facilitates the hydrolysis of β-glycosidic bonds between oligosaccharides and ceramides. Additionally, it catalyzes transglycosylation reactions, transferring the sugar moiety of glycosphingolipids to the primary hydroxyl groups of various 1-alkanols.

Recombinant endoglycoceramidase II assisted by activator II is a biocatalyst and a key enzyme in new biocatalysis technology. Enzyme engineering focuses on improving the reaction kinetics, substrate selectivity, and activity under extreme conditions (such as low or high pH). By introducing stimulus-responsive modifications to these enzymes, dynamic control of activity becomes feasible.

PFB-FDGlu is a lysosomal Glucocerebrosidase (GCase) substrate with cell-permeable specificity that is cleaved to produce fluorescein.PFB-FDGlu is often used in conjunction with a flow cytometer to measure GCase activity in living cells on an individual cell basis.

4-Methylumbelliferyl-β-D-Glucopyranoside (4-MU-GLU) is used in the GCase activity assay based on the catalytic hydrolysis of 4-methylumbelliferyl β-D-glucopyranoside that releases the highly fluorescent 4-methylumbelliferyl (4-MU).

AMP-Deoxynojirimycin (AMP-DNM) is a potent inhibitor of ceramide glucosyltransferase and GCase 2, as well as GlcCer biosynthesis, and is utilized in the study of Parkinson's disease and diabetes.

GT-02216 is capable of binding allosterically to GCase, thereby enhancing its function. In primary human fibroblasts, it increases GCase activity and decreases Tau accumulation in mutant GBA1 fibroblasts. Additionally, GT-02216 offers protection to hippocampal primary neurons exposed to Tau oligomer in a rat model.

Afegostat is an acid β-glucosidase (GCase) active site inhibitor that acts as a pharmacological chaperone. Afegostat inhibits GCase with K(i) ~30 nM for wild-type and mutant enzymes. Afegostat was an experimental drug for the treatment of certain forms of

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. Due to GCase involvement in pathological disorders consequent to enzyme deficiency, Glucocerebrosidase-IN-1 can be used for research of Gaucher disease (GD) and Parkinson's disease (PD)[1].

Afegostat, aslo known as Isofagomine, and AT2101, was an experimental drug for the treatment of certain forms of Gaucher's disease. Isofagomine (IFG) is an acid β-glucosidase (GCase) active site inhibitor that acts as a pharmacological chaperone. FG inhibits GCase with K(i) ~30 nM for wild-type and mutant enzymes (N370S and V394L). Administration of IFG (30 mg/kg/day) to the mice homozygous for GCase mutations (V394L, D409H, or D409V) led to increased GCase activity in visceral tissues and brain extracts.

Glucocerebrosidase-IN-1 (compound 11a) hydrochloride is a potent and selective glucocerebrosidase (GCase) inhibitor with IC50 of 29.3 μM and Ki of 18.5 μM, used in the research of Gaucher disease (GD) and Parkinson's disease (PD) [1].