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Results for "

Gaucher disease

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Autophagy (5) Glucosidase (10) Glucosylceramide Synthase (GCS) (10) Isotope-Labeled Compounds (3)
By Research Areas:	Inflammation/Immunology (2) Metabolic Disease (15) Neurological Disease (15)
Click Chemistry:	Alkynes (1)

Inhibitors & Agonists

Natural
Products

Isotope-Labeled Compounds

Click Chemistry

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-N7745

Glucosylsphingosine Glucopsychosine; Lyso-Gb1	Others	Metabolic Disease
Glucosylsphingosine (lyso-Gb1) is a deacylated form of glucosylceramide and is also degraded by the glucocerebrosidase. Glucosylsphingosine is a very promising, reliable and specific biomarker for monitoring Gaucher disease .

HY-17020

Miglustat 3 Publications Verification N-Butyldeoxynojirimycin; NB-DNJ; OGT 918	Glucosylceramide Synthase (GCS)	Neurological Disease Inflammation/Immunology
Miglustat (N-Butyldeoxynojirimycin) is an orally active and reversible ceramide glucosyltransferase inhibitor. Miglustat can be used for the research of type I gaucher disease .

HY-17020A

Miglustat hydrochloride 3 Publications Verification N-Butyldeoxynojirimycin hydrochloride; NB-DNJ hydrochloride; OGT 918 hydrochloride	Glucosylceramide Synthase (GCS)	Neurological Disease Inflammation/Immunology
Miglustat (N-Butyldeoxynojirimycin) hydrochloride is an orally active and reversible ceramide glucosyltransferase inhibitor. Miglustat hydrochloride can be used for the research of type I gaucher disease .

HY-147976

Glucocerebrosidase-IN-1	Glucosidase	Neurological Disease Metabolic Disease
Glucocerebrosidase-IN-1 (compound 11a) is a potent and selective GCase (glucocerebrosidase) inhibitor, with an IC₅₀ of 29.3 μM and a K_i of 18.5 μM. Glucocerebrosidase-IN-1 can be used for the research of Gaucher disease (GD) and Parkinson’s disease (PD) .

HY-147976A

Glucocerebrosidase-IN-1 hydrochloride	Glucosidase	Neurological Disease
Glucocerebrosidase-IN-1 (compound 11a) hydrochloride is a potent and selective GCase (glucocerebrosidase) inhibitor, with an IC₅₀ of 29.3 μM and a K_i of 18.5 μM. Glucocerebrosidase-IN-1 hydrochloride can be used for the research of Gaucher disease (GD) and Parkinson’s disease (PD) .

HY-126362

ML266	Glucosidase	Others
ML266 is glucocerebrosidase (GCase) molecule chaperone with IC₅₀ of 2.5 µM. ML266 binds to GCase and transports of the mutant protein to the lysosome, and resume the activity of GCase. ML266 dose not inhibit the GCase enzyme’s action. ML266 has the potential for the research of gaucher disease .

HY-B1039

Ambroxol 2 Publications Verification NA-872	Glucosidase Autophagy	Neurological Disease Metabolic Disease
Ambroxol (NA-872), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research .

HY-B1039A

Ambroxol hydrochloride 2 Publications Verification NA-872 hydrochloride	Glucosidase Autophagy	Neurological Disease Metabolic Disease
Ambroxol hydrochloride (NA-872 hydrochloride), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol hydrochloride is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol hydrochloride induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research .

HY-B1039C

Ambroxol (acefylline) NA-872 (acefylline); Acebrophylline	Glucosidase Autophagy	Neurological Disease Metabolic Disease
Ambroxol (NA-872) acefylline, an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol acefylline is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol acefylline induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research .

HY-117861

ML198	Glucosidase	Metabolic Disease
ML198 is a glucocerebrosidase (GCase) modulator with an EC₅₀ of 0.4 μM. ML198 is an activator and non-inhibitory chaperone of glucocerebrosidase. ML198 can be used for the research of Gaucher disease . ML198 is a click chemistry reagent, it contains an Alkyne group and can undergo copper-catalyzed azide-alkyne cycloaddition (CuAAc) with molecules containing Azide groups.

HY-108721

Velaglucerase alfa	Others	Metabolic Disease
Velaglucerase alfa is produced through gene activation technology and is identical to wild-type enzyme. Velaglucerase alfa targets accumulated glucocerebroside primarily within the lysosome of the macrophages in the affected organs and systems. Velaglucerase alfa is an enzyme replacement therapy (ERT) for type 1 Gaucher disease (GD) .

HY-153592

Therapeutic agent-1

Others Others

Therapeutic agent-1 is a heteroaryl compound that can be used in Gaucher disease glucocerebrosidase activity enzyme replacement therapy .

Therapeutic agent-1	Others	Others
Therapeutic agent-1 is a heteroaryl compound that can be used in Gaucher disease glucocerebrosidase activity enzyme replacement therapy .

HY-16743

Ibiglustat 2 Publications Verification Venglustat; SAR402671; GZ402671	Glucosylceramide Synthase (GCS)	Metabolic Disease
Ibiglustat (Venglustat) is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease .

HY-110363

Miglustat-d9 hydrochloride N-Butyldeoxynojirimycin-d₉ hydrochloride; NB-DNJ-d₉ hydrochloride; OGT 918-d₉ hydrochloride	Isotope-Labeled Compounds Glucosylceramide Synthase (GCS)	Neurological Disease
Miglustat-d₉ (hydrochloride) is the deuterium labeled Miglustat (hydrochloride). Miglustat hydrochloride is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1)[1][2].

HY-16743B

Ibiglustat succinate 2 Publications Verification Venglustat succinate; SAR402671 succinate; GZ402671 succinate	Glucosylceramide Synthase (GCS)	Neurological Disease
Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat succinate can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease .

HY-17020S

Miglustat-d9 N-Butyldeoxynojirimycin-d₉; NB-DNJ-d₉; OGT 918-d₉	Isotope-Labeled Compounds Glucosylceramide Synthase (GCS)	Neurological Disease
Miglustat-d9 is the deuterium labeled Miglustat. Miglustat (N-Butyldeoxynojirimycin) is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1) .

HY-16743A

Ibiglustat (L-Malic acid) 2 Publications Verification Venglustat (L-Malic acid); SAR402671 (L-Malic acid); GZ402671 (L-Malic acid)	Glucosylceramide Synthase (GCS)	Metabolic Disease
Ibiglustat (Venglustat) L-Malic acid is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat L-Malic acid can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease .

HY-B1039AS

Ambroxol-d5 hydrochloride NA-872-d₅ hydrochloride	Isotope-Labeled Compounds Glucosidase Autophagy	Neurological Disease Metabolic Disease
Ambroxol-d₅ (hydrochloride) is the deuterium labeled Ambroxol hydrochloride. Ambroxol hydrochloride (NA-872 hydrochloride), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol hydrochloride is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol hydrochloride induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research[1][2].

HY-B1039AR

Ambroxol hydrochloride (Standard) NA-872 hydrochloride (Standard)	Glucosidase Autophagy	Neurological Disease Metabolic Disease
Ambroxol (hydrochloride) (Standard) is the analytical standard of Ambroxol (hydrochloride). This product is intended for research and analytical applications. Ambroxol hydrochloride (NA-872 hydrochloride), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol hydrochloride is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol hydrochloride induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research .

HY-144270

Glucosylceramide synthase-IN-3	Glucosylceramide Synthase (GCS)	Neurological Disease Metabolic Disease
Glucosylceramide synthase-IN-3 (compound BZ1) is a potent, brain-penetrant and orally active glucosylceramide synthase (GCS) inhibitor with IC₅₀s of 16 nM for human GCS.Glucosylceramide synthase-IN-3 can be used for Gaucher's disease research .

HY-144266

TP-060 Glucosylceramide synthase-IN-1	Glucosylceramide Synthase (GCS)	Neurological Disease Metabolic Disease
Glucosylceramide synthase-IN-1 (T-036) a potent, brain-penetrant and orally active glucosylceramide synthase (GCS) inhibitor with IC₅₀s of 31 nM and 51 nM for human GCS and mouse GCS, respectively. Glucosylceramide synthase-IN-1 can be used for Gaucher's disease research .

HY-114043

NCGC00092410	Glucosidase	Metabolic Disease
NCGC00092410 is a potent, selective, and nonsugar glucocerebrosidase (GC) inhibitor, with an IC₅₀ of 31 nM. NCGC00092410 shows no activity against the related hydrolases at concentrations up to 77 μM. NCGC00092410, a GC chaperone, and increases the activity and lysosomal localization of glucocerebrosidase in mutant cell lines. NCGC00092410 can be used for the research of Gaucher disease .

HY-144267

Glucosylceramide synthase-IN-2	Glucosylceramide Synthase (GCS)	Neurological Disease Metabolic Disease
Glucosylceramide synthase-IN-2 (compound T-690) is a potent, brain-penetrant and orally active glucosylceramide synthase (GCS) inhibitor with IC₅₀s of 15 nM and 190 nM for human GCS and mouse GCS, respectively.Glucosylceramide synthase-IN-2 exhibits noncompetitive type inhibition with C8-ceramide and UDP-glucose.Glucosylceramide synthase-IN-2 can be used for Gaucher's disease research .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-N7745

Glucosylsphingosine Glucopsychosine; Lyso-Gb1	Structural Classification Classification of Application Fields Animals Source classification Metabolic Disease Endogenous metabolite Disease Research Fields Saccharides	Others
Glucosylsphingosine (lyso-Gb1) is a deacylated form of glucosylceramide and is also degraded by the glucocerebrosidase. Glucosylsphingosine is a very promising, reliable and specific biomarker for monitoring Gaucher disease .

Cat. No.	Product Name	Chemical Structure

HY-B1039AS

Ambroxol-d5 hydrochloride
Ambroxol-d₅ (hydrochloride) is the deuterium labeled Ambroxol hydrochloride. Ambroxol hydrochloride (NA-872 hydrochloride), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol hydrochloride is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol hydrochloride induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research[1][2].

Ambroxol-d5 hydrochloride

Ambroxol-d₅ (hydrochloride) is the deuterium labeled Ambroxol hydrochloride. Ambroxol hydrochloride (NA-872 hydrochloride), an active metabolite of the proagent Bromhexine, has potent expectorant effects. Ambroxol hydrochloride is a glucocerebrosidase (GCase) chaperone and increases glucocerebrosidase activity. Ambroxol hydrochloride induces lung autophagy and has the potential for Parkinson disease and neuronopathic Gaucher disease research[1][2].

HY-110363

Miglustat-d9 hydrochloride
Miglustat-d₉ (hydrochloride) is the deuterium labeled Miglustat (hydrochloride). Miglustat hydrochloride is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1)[1][2].

HY-17020S

Miglustat-d9
Miglustat-d9 is the deuterium labeled Miglustat. Miglustat (N-Butyldeoxynojirimycin) is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1) .

ML198		Alkynes
ML198 is a glucocerebrosidase (GCase) modulator with an EC₅₀ of 0.4 μM. ML198 is an activator and non-inhibitory chaperone of glucocerebrosidase. ML198 can be used for the research of Gaucher disease . ML198 is a click chemistry reagent, it contains an Alkyne group and can undergo copper-catalyzed azide-alkyne cycloaddition (CuAAc) with molecules containing Azide groups.

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