gcs

GCS-12, a Th1 2-balanced sulfonamide glycolipid, enhances its interaction with CD1d. It acts as an agonist for Natural Killer T cells (NKT cells), inducing the secretion of cytokines IFN-γ and IL-4. In mice, it has demonstrated immunoregulatory and antitumor activities.

GCS-11 is an effective activator of natural killer T (NKT) cells. It specifically promotes the production of IFN-γ and IL-4, with a particular selectivity for IFN-γ. As a compound, GCS-11 exhibits anticancer activity and holds potential as a promising adjuvant in cancer immunotherapy or vaccine development.

Sinbaglustat (OGT2378) (OGT2378) is a dual inhibitor of glucose ceramide synthetase (GCS) and non-lysosomal glucose ceramidase (GBA2).Sinbaglustat is an orally administered N-alkyl iminosugar that crosses the blood-brain barrier.Sinbaglustat is used for the treatment of lysosomal storage disorders and for the study of central neurodegenerative disorders associated with lysosomal dysfunction. Sinbaglustat is used for the treatment of lysosomal storage disorders and for the study of lysosomal dysfunction-related central neurodegenerative diseases.

Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthesis of GL-3. Ibiglustat is potentially useful for treating Fabry disease. Fabry disease is a rare lysosomal storage disorder, which results in abnormal tissue deposits of a particular fatty substance called globotriaosylceramide (GL-3 or Gb3) throughout the body.

Miglustat hydrochloride (N-Butyldeoxynojirimycin hydrochloride) is an inhibitor of glucosylceramide synthase and can be used for studies about Type I Gaucher disease.

Lucerastat（NBDGJ） is an orally available inhibitor of Glucosylceramide Synthase (GCS) with therapeutic potential for Fabry disease. GCS is a key enzyme in sphingolipid synthesis and inhibition of its activity reduces the accumulation of harmful substrates.

Eliglustat (Genz 99067) is an oral inhibitor of glucosylceramide synthase which is used in the therapy of type 1 Gaucher disease.

Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant inhibitor of glucosylceramide synthase (GCS) utilized in the investigation of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease.

L-Buthionine-(S,R)-sulfoximine hydrochloride is a potent, cell-permeable, fast-acting, irreversible inhibitor of G-glutamylcysteine synthetase (γ-GCS) that depletes cellular glutathione levels. It has IC50s of 1.9 μM, 8.6 μM, and 29 μM in melanoma, breast, and ovarian tumor specimens, respectively [1] [2].

Glucosylceramide synthase-IN-3 (compound BZ1) is a highly potent, brain-penetrant, and orally active inhibitor of glucosylceramide synthase (GCS), with an IC50 value of 16 nM for human GCS, and has potential applications in Gaucher's disease research [1] [2].

R-HP210 inhibits LPS-induced transcription of several pro-inflammatory genes (IL-1β, IL-6 and COX-2). r-HP210 has an IC50 value of 3.80 μM for NF-κB-mediated bolus trans-suppression. r-HP210 does not induce trans-activation of glucocorticoids (GCs).

Glucosylceramide synthase-IN-2 (compound T-690) is a potent, blood-brain barrier-permeable, orally active inhibitor of glucosylceramide synthase (GCS), with IC50 values of 15 nM for human GCS and 190 nM for mouse GCS. It exhibits non-competitive inhibition of C8 ceramide and UDP glucose.

Glucosylceramide synthase-IN-1 (T-036) is a potent, orally active, blood-brain barrier permeable glucosylceramide synthase (GCS) inhibitor, acting on human GCS (IC50: 31 nM) and mouse GCS (IC50: 51 nM), and can be used to study Gaucher disease.

CCG-203586 is a novel potent glucosylceramide synthase (GCS) inhibitor.

D-threo-PDMP is a potent inhibitor of glucoceramide synthase (GCS), effectively reducing cell surface glycosphingolipids by inhibiting glycosylation. Additionally, it decreases the axon plexus's total length and the number of axon branch points, ultimately inhibiting neurite growth.

Glucosylceramide Synthase-IN-4 (compound 12) serves as a potent inhibitor of glucosylceramide synthase (GCS), exhibiting an IC50 of 6.8 nM. It demonstrates superior pharmacokinetic properties and robust stability in human hepatocytes. Additionally, this compound is noted for its effective CNS penetration and acceptable PXR selectivity [1].