β-glucocerebrosidase

NCGC00092410 (ML008) is a glucocerebrosidase (GC) inhibitor with IC50 value of 31 nM. It can be used for the study of Gaucher disease caused by mutation of glucocerebrase gene.

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.

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.

ML266, a glucocerebrosidase (GCase) chaperone with an IC50 of 2.5 µM, facilitates the transport of the mutant protein to the lysosome, thereby restoring GCase activity without inhibiting the enzyme's function. This compound shows promise for Gaucher disease research.

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.

13C6 Glucosylsphingosine (d18:1) is an isotopically enriched form of 1-β-D-glucosylsphingosine (d18:1) that is intended for use as an internal standard for the quantification of 1-β-D-glucosylsphingosine by GC- or LC-MS. 1-β-D-Glucosylsphingosine is a lysolipid derivative of glucosylcerebroside that decreases activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner.

1-β-D-Glucosylsphingadienine is a glucosylsphingosine, which are deacetylated lysolipid derivatives of glucosylcerebrosides . They are formed when sphingosines undergo glucosidation by UDP-glucose. Glucosylsphingosines completely reduce neurite outgrowth and induce death of LA-N-2 cells at concentrations of 10 and 50 μM, respectively. They also decrease the activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner. Glucosylsphingosine levels are elevated in patients with Gaucher's disease, both in the spleen (in types 1, 2, and 3) and brain (type 2 and 3); thus, glucosylsphingosine has been used as a key biomarker of the disease. This product contains 1-β-D-glucosylsphingadienine isolated from plants.

Glucosylsphingosine (Lyso-Gb1) is a naturally occurring glycosyl ceramide, a metabolite of GBA (acid β-glucosidase), involved in cellular recognition, signaling, and intercellular interactions.Glucopsychosine accumulates in certain neurodegenerative diseases (e.g., Gaucher disease), disrupts lysosomal function through activation of mammalian target of rapamycin complex 1 (mTORC1), and is a noncompetitive inhibitor of glucocerebrosidase.

HPSE1-IN-1 (compound 16) functions as a selective Heparanase-1 (HPSE1) inhibitor, displaying moderate activity against exo-β-d-glucuronidase (GUSβ) and glucocerebrosidase (GBA) [1].

HPSE1-IN-2 (compound 2) functions as an inhibitor of Heparanase-1 (HPSE1) while concurrently inhibiting exo-β-d-glucuronidase (GUSβ) and glucocerebrosidase (GBA) activities [1].

Conduritol B epoxide is an irreversible inhibitor of β-glucosidase (GCase).