glucosylceramide synthase

G43 is a potent and selective glucosyltransferase inhibitor that inhibits GtfB and GtfC with Kd values of 3.7 μM and 46.9 nM, respectively.G43 exhibits in vitro and in vivo anti-Streptococcus mutans activity and can be used for caries studies.

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].

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.

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].

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.

Genz-123346 blocks the conversion of ceramide to glucosylceramide (GL1) and inhibits GM1 with an IC50 value of 14 nM. Genz-123346 is a potent, orally available glucosylceramide synthase inhibitor.

Ibiglustat (L-Malic acid) (Ibiglustat L-Malic acid) is a selective, brain-penetrant, and allosteric inhibitor of glucosylceramide synthase. Ibiglustat (L-Malic acid) can be used in studies about PD Parkinson's disease, SRT in Fabry's and Gaucher's.

Miglustat (NB-DNJ) is an alkylated product of imino sugar deoxynojirimycin.It is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1).

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.

(-)-L-threo-PDMP is an enhancer of ganglioside biosynthesis. The (-)-L-threo-PDMP (1S,2S) isomer is the active stimulatory component of DL-threo-PDMP in contrast to (+)-D-threo-PDMP , which is an inhibitor of glucosylceramide synthetase. (-)-L-threo-PDMP upregulates GM3, GD3, and GQ1b synthase levels, which are glycosyltransferases involved in ganglioside biosynthesis, and increases ganglioside levels in cells. It increases neurite outgrowth and synapse formation in vitro and improves retention of a long-term memory learned prior to forebrain ischemia in rats when administered following ischemia at a dose of 40 mg/kg per day.

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.

PDMP is a ceramide analog first prepared in a search for inhibitors of glucosylceramide synthase. PDMP has two adjacent chiral centers (C1 and C2) allowing for the formation of four possible isomers. PDMP contains all four of these stereoisomers. PDMP inhibits glucosylceramide synthase by 90% when used at a concentration of 0.8 μM in MDCK cell homogenates, however, the ability to inhibit glucosylceramide synthase has been found to reside in the D-threo (1R,2R) enantiomer. The D-threo PDMP enantiomer is also responsible for inhibition of β-1,4-galactosyltransferase 6 and prevention of lactosylceramide synthesis, which is a promotor of neuroinflammation in mice during chronic experimental autoimmune encephalomyelitis (EAE), a model of multiple sclerosis. PDMP enhances curcumin-induced inhibition of proliferation, JNK activation, and Akt inhibition, as well as induction of apoptosis in WM-115 melanoma cells in vitro.

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

D-threo-PPMP is a glucosylceramide (GlyCer) synthetase inhibitor.1,2It is the active enanantiomer and enzymatic inhibitory component of the racemic DL-threo-PPMP . In MDCK kidney epithelial cells, D-threo-PPMP induces a 70% reduction in cell growthin vitroat 20 μM and significantly inhibits DNA synthesis at 3 μM.3[Matreya, LLC. Catalog No. 1865] 1.Shen, W., Henry, A.G., Paumier, K.L., et al.Inhibition of glucosylceramide synthase stimulates autophagy flux in neuronsJ. Neurochem.129(5)884-894(2014) 2.Lee, L., Abe, A., and Shayman, J.A.Improved inhibitors of glucosylceramide synthaseJ. Biol. Chem.274(21)14662-14669(1999) 3.Abe, A., Inokuchi, J.-i., Jimbo, M., et al.Improved inhibitors of glucosylceramide synthaseJ. Biochem.111(2)191-196(1992)

(+)-D-threo-PDMP is a ceramide analog and is one of the four possible stereoisomers of PDMP . (+)-D-threo-PDMP is an inhibitor of glucosylceramide synthase. It inhibits glucosylceramide synthase by 50% when used at a concentration of 5 μM in an enzyme assay. (+)-D-threo-PDMP is the active component of racemic DL-threo-PDMP .

Deoxynojirimycin (dNM) tetrabenzyl ether is an intermediate for the synthesis of glucosylceramide synthase inhibitors such as 1-dNM, a glucose analog that potently inhibits α-glucosidase I and II.

D-threo-PPMP, a powerful glucosylceramide (GlcCer) synthase inhibitor, effectively impedes karyokinesis and decreases cyst formation.

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.

Ibiglustat hydrochloride is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor.

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

Miglustat-d 9 (hydrochloride), a deuterium-labeled variant of Miglustat (hydrochloride), functions as a glucosylceramide synthase inhibitor. Its primary application is in the treatment of Type I Gaucher disease (GD1) [1][2].