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Results for "

Niemann-Pick

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Amino Acid Derivatives (1) Autophagy (6) Bacterial (1) Beta-lactamase (1) Drug Metabolite (1) Endogenous Metabolite (2) Filovirus (1) Fluorescent Dye (1) HSP (3) Isotope-Labeled Compounds (1) Keap1-Nrf2 (6)
By Research Areas:	Cancer (7) Cardiovascular Disease (7) Infection (3) Inflammation/Immunology (5) Metabolic Disease (6) Neurological Disease (7)

Inhibitors & Agonists

Natural
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Recombinant Proteins

Isotope-Labeled Compounds

Antibodies

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-100353

C12-Ceramide N-Lauroyl-D-erythro-sphingosine; N-Laurylsphingosine	Others	Metabolic Disease Cancer
C12-Ceramide (N-Lauroyl-D-erythro-sphingosine), a naturally occurring ceramide, is formed by hydrolysis of C12 sphingomyelin. C12-Ceramide can enhance the Doxorubicin toxicity in MDA-MB-231 cells. C12-Ceramide also can be used to diagnose types A and B Niemann-Pick disease .

HY-W395779

EBOV-IN-1	Filovirus	Infection
EBOV-IN-1 (com 3.47) is an adamantane dipeptide piperazine and an inhibitor of Ebola virus (EBOV). EBOV-IN-1 targets Niemann-Pick C1 (NPC1) and inhibits its binding to the EBOV glycoprotein (GP) that activates and mediates viral penetration into host cells, thereby inhibiting EBOV infection. EBOV-IN-1 inhibits pseudotyped EBOV infection with an IC50 of 13 nM .

HY-17376

Ezetimibe Maximum Cited Publications 8 Publications Verification SCH 58235	Keap1-Nrf2 Autophagy	Cardiovascular Disease Cancer
Ezetimibe (SCH 58235) is a potent cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

HY-17376S

Ezetimibe-d4 SCH 58235-d₄	Autophagy Keap1-Nrf2 Isotope-Labeled Compounds	Cardiovascular Disease Cancer
Ezetimibe-d₄ is the deuterium labeled Ezetimibe. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

HY-17376S1

Ezetimibe-d4-1 SCH 58235-d₄-1	Keap1-Nrf2 Autophagy	Cardiovascular Disease Cancer
Ezetimibe-d₄-1 is deuterium labeled Ezetimibe. Ezetimibe (SCH 58235) is a potent cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

HY-133114

Ezetimibe ketone EZM-K	Keap1-Nrf2 Autophagy	Cardiovascular Disease Cancer
Ezetimibe ketone (EZM-K) is a phase-I metabolite of Ezetimibe. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator. Ezetimibe is a potent cholesterol absorption inhibitor .

HY-17376R

Ezetimibe (Standard) Maximum Cited Publications 8 Publications Verification SCH 58235 (Standard)	Keap1-Nrf2 Autophagy	Cardiovascular Disease Cancer
Ezetimibe (Standard) is the analytical standard of Ezetimibe. This product is intended for research and analytical applications. Ezetimibe (SCH 58235) is a potent cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

HY-W414824

3β,7β-Dihydroxy-5-cholestenoic acid	Others	Neurological Disease
3β,7β-Dihydroxy-5-cholestenoic acid is a C27 acid that displays elevated levels in Niemann-Pick type C and Niemann-Pick type B diseases, contributing to toxicity in oculomotor neurons. It is synthesized from 3β-hydroxy-7-oxocholest-5-en-(25R)26-oic acid (3βH,7O-CA) through the enzymatic action of hydroxysteroid 11-β dehydrogenase 1.

HY-135388

ent-Ezetimibe ent-SCH 58235	Drug Metabolite	Cardiovascular Disease
ent-Ezetimibe (ent-SCH 58235) is the RRS-enantiomer of Ezetimibe. Ezetimibe is a potent cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator .

HY-136059

Desfluoro-ezetimibe	Keap1-Nrf2 Autophagy	Cardiovascular Disease Cancer
Desfluoro-ezetimibe is a desfluoro impurity of Ezetimibe. Ezetimibe is a potent, metabolically stable cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator .

HY-59291

Levacetylleucine	Endogenous Metabolite Amino Acid Derivatives	Neurological Disease Metabolic Disease Inflammation/Immunology
Levacetylleucine, an orally bioavailable and brain-penetrant endogenous metabolite, is an acetylated derivative of amino acid leucine. Levacetylleucine is the active form of N-acetyl-leucine (NAL). Levacetylleucine attenuates neuronal death and neuroinflammation in the cortical tissue of mice. Levacetylleucine also potentially improves ameliorates lysosomal and metabolic dysfunction. Levacetylleucine improves compensation of postural symptoms after unilateral chemical labyrinthectomy (UL) in rats. Levacetylleucine is promising for research of neurological manifestations of Niemann-Pick disease type C, traumatic brain injury and neurodegeneration prevention .

HY-113498

Sphingomyelin	Endogenous Metabolite	Neurological Disease Metabolic Disease Inflammation/Immunology
Sphingomyelin is a eukaryotic sphingolipid and one of the major constituents of cell membranes and particularly abundant in the myelin sheath that surrounds neuronal axons. Sphingomyelin plays an important role in cell processes, the regulation of inflammatory responses, and signal transduction. Sphingomyelin metabolism is associated with various central nervous system diseases and Niemann–Pick disease .

HY-106443A

Arimoclomol maleate 3 Publications Verification BRX-220	HSP	Neurological Disease Metabolic Disease Inflammation/Immunology
Arimoclomol maleate (BRX-220) is an orally active co-inducer of heat shock proteins (HSP) . Arimoclomol protects motor neurons by enhancing Hsp expression, thus directly affecting protein aggregation and clearance of misfolded assemblies via the proteasome-ubiquitin system .Arimoclomol maleate can be used for the study of Niemann–Pick disease type C.

HY-106443

Arimoclomol BRX-220 free base	HSP	Neurological Disease Metabolic Disease Inflammation/Immunology
Arimoclomol (BRX-220 free base) is an orally active co-inducer of heat shock proteins (HSP) . Arimoclomol protects motor neurons by enhancing Hsp expression, thus directly affecting protein aggregation and clearance of misfolded assemblies via the proteasome-ubiquitin system .Arimoclomol can be used for the study of Niemann–Pick disease type C.

HY-106443B

Arimoclomol citrate BRX-220 citrate	HSP	Neurological Disease Metabolic Disease Inflammation/Immunology
Arimoclomol citrate (BRX-220 citrate) is an orally active co-inducer of heat shock proteins (HSP) . Arimoclomol citrate protects motor neurons by enhancing Hsp expression, thus directly affecting protein aggregation and clearance of misfolded assemblies via the proteasome-ubiquitin system . Arimoclomol citrate can be used for the study of Niemann–Pick disease type C.

HY-116815

Lalistat 1	Beta-lactamase Bacterial	Infection Neurological Disease
Lalistat 1 is a potent, selective, and competitive inhibitor of lysosomal acid lipase (LAL) and against purified human LAL (phLAL) with an IC₅₀ of 68 nM. Lalistat 1 is a inhibitor of immunoglobulin A1 protease (IgA1P) proteases for H. influenzae, has less effects on other serine hydrolases (trypsin or β-lactamase, etc.). Lalistat 1 can be used for the research of niemann-pick type C (NPC) disease .

HY-145549

C20 Sphingomyelin (d18:1/20:0) N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine; SM(d18:1/20:0)	Others	Infection
C20 Sphingomyelin is a naturally occurring sphingolipid. Levels of C20 sphingomyelin are upregulated in the hippocampus of rats with diabetes induced by streptozotocin (HY-13753) and in human plasma where it is positively correlated with insulin resistance in obese humans. C20 sphingomyelin is also upregulated in the liver of a mouse model of Niemann-Pick type C1 disease, a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder. The plasma concentration of C20 sphingomyelin is decreased in men with prostate cancer. As this product is derived from a natural source, there may be variations in the sphingoid backbone.

HY-131682

3-C6-NBD-cholesterol 3-Hexanoyl-NBD-cholesterol	Fluorescent Dye	Others
3-C6-NBD-cholesterol is a fluorescent analog of Chol that can be used to measure the kinetics of membrane and intracellular trafficking .

Levacetylleucine	Structural Classification Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite Amino Acid Derivatives
Levacetylleucine, an orally bioavailable and brain-penetrant endogenous metabolite, is an acetylated derivative of amino acid leucine. Levacetylleucine is the active form of N-acetyl-leucine (NAL). Levacetylleucine attenuates neuronal death and neuroinflammation in the cortical tissue of mice. Levacetylleucine also potentially improves ameliorates lysosomal and metabolic dysfunction. Levacetylleucine improves compensation of postural symptoms after unilateral chemical labyrinthectomy (UL) in rats. Levacetylleucine is promising for research of neurological manifestations of Niemann-Pick disease type C, traumatic brain injury and neurodegeneration prevention .

Sphingomyelin	Structural Classification Neurological Disease Classification of Application Fields Ketones, Aldehydes, Acids Source classification Endogenous metabolite Inflammation/Immunology Disease Research Fields	Endogenous Metabolite
Sphingomyelin is a eukaryotic sphingolipid and one of the major constituents of cell membranes and particularly abundant in the myelin sheath that surrounds neuronal axons. Sphingomyelin plays an important role in cell processes, the regulation of inflammatory responses, and signal transduction. Sphingomyelin metabolism is associated with various central nervous system diseases and Niemann–Pick disease .

Species:	Human (2) Rat (1) Mouse (1)
Tag:	His (4)
Source:	HEK293 (4)

Cat. No.	Compare	Product Name	Species	Source

HY-P74695

	Niemann Pick C1/NPC1 Protein, Human (His) NPC intracellular cholesterol transporter 1; Niemann-Pick C1 protein; NPC1	Human	HEK293
	The Niemann Pick C1/NPC1 protein is an intracellular cholesterol transporter that cooperates with NPC2 to promote cholesterol efflux from the endosomal/lysosomal compartment. NPC2 transfers unesterified cholesterol to the N-terminal domain of NPC1, where the cholesterol is bound in a hydrophobic pocket. Niemann Pick C1/NPC1 Protein, Human (His-Flag) is the recombinant human-derived Niemann Pick C1/NPC1 protein, expressed by HEK293 , with N-His labeled tag. The total length of Niemann Pick C1/NPC1 Protein, Human (His-Flag) is 251 a.a., with molecular weight of ~38-55 kDa.

HY-P74692

	Niemann Pick C2/NPC2 Protein, Rat (HEK293, His) NPC intracellular cholesterol transporter 2; Niemann-Pick disease type C2 protein; HE1	Rat	HEK293
	Niemann-Pick C2/NPC2 protein is an important member of the NPC2 family and plays an important role in lipid metabolism and transport, helping to regulate intracellular cholesterol homeostasis and lipid transport.Its involvement in cellular processes highlights its importance in maintaining lipid homeostasis.Niemann Pick C2/NPC2 Protein, Rat (HEK293, His) is the recombinant rat-derived Niemann Pick C2/NPC2 protein, expressed by HEK293 , with C-His labeled tag.

HY-P74693

	Niemann Pick C2/NPC2 Protein, Mouse (HEK293, His) NPC intracellular cholesterol transporter 2; Niemann-Pick disease type C2 protein; HE1	Mouse	HEK293
	Niemann Pick C2/NPC2 Protein, a lysosomal protein, plays a critical role in cholesterol trafficking and lipid metabolism. It facilitates the transport of cholesterol from lysosomes to other cellular compartments. Understanding the functions of NPC2 Protein is vital for studying lipid disorders and developing therapeutic strategies targeting abnormal cholesterol metabolism. Niemann Pick C2/NPC2 Protein, Mouse (HEK293, His) is the recombinant mouse-derived Niemann Pick C2/NPC2 protein, expressed by HEK293 , with C-His labeled tag. The total length of Niemann Pick C2/NPC2 Protein, Mouse (HEK293, His) is 130 a.a., with molecular weight of ~15.9-19 kDa.

HY-P74694

	Niemann Pick C2/NPC2 Protein, Human (HEK293, His) NPC intracellular cholesterol transporter 2; Niemann-Pick disease type C2 protein; HE1	Human	HEK293
	The Niemann Pick C2/NPC2 Protein is a crucial lysosomal protein that regulates cholesterol trafficking and lipid metabolism. It enables the movement of cholesterol from lysosomes to other parts of the cell. Studying the functions of NPC2 Protein is essential for understanding lipid disorders and developing treatments targeting disrupted cholesterol metabolism. Niemann Pick C2/NPC2 Protein, Human (HEK293, His) is the recombinant human-derived Niemann Pick C2/NPC2 protein, expressed by HEK293 , with C-His labeled tag.

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HY-17376S

Ezetimibe-d4
Ezetimibe-d₄ is the deuterium labeled Ezetimibe. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

HY-17376S1

Ezetimibe-d4-1
Ezetimibe-d₄-1 is deuterium labeled Ezetimibe. Ezetimibe (SCH 58235) is a potent cholesterol absorption inhibitor. Ezetimibe is a Niemann-Pick C1-like1 (NPC1L1) inhibitor, and is a potent Nrf2 activator.

Cat. No.	Compare	Product Name	Application	Reactivity

HY-P82545

	Niemann Pick C1 Antibody (YA2290) Niemann Pick C1 protein precursor; NPC; NPC1	WB, IHC-P, ICC/IF, FC	Human, Mouse, Rat
	Niemann Pick C1 Antibody (YA2290) is a rabbit-derived non-conjugated IgG antibody (Clone NO.: YA2290), targeting Niemann Pick C1, with a predicted molecular weight of 142 kDa (observed band size: 180 kDa). Niemann Pick C1 Antibody (YA2290) can be used for WB, IHC-P, ICC/IF, FC experiment in human, mouse, rat background.

HY-P81654

	Niemann Pick C2 Antibody (YA1399) HE1; EDDM1	WB, IHC-P	Human, Mouse, Rat
	Niemann Pick C2 Antibody (YA1399) is a rabbit-derived non-conjugated IgG antibody (Clone NO.: YA1399), targeting Niemann Pick C2, with a predicted molecular weight of 17 kDa (observed band size: 17 kDa). Niemann Pick C2 Antibody (YA1399) can be used for WB, IHC-P experiment in human, mouse, rat background.

HY-P81911

	Niemann Pick C1 Like 1 Antibody (YA1656) NPC1 LIKE 1; NPC1L1	WB	Human
	Niemann Pick C1 Like 1 Antibody (YA1656) is a biotin-conjugated non-conjugated IgG antibody, targeting Niemann Pick C1 Like 1, with a predicted molecular weight of 149 kDa. Niemann Pick C1 Like 1 Antibody (YA1656) can be used for WB experiment in human background.

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Niemann-Pick

Inhibitors & Agonists

NaturalProducts

Recombinant Proteins

Isotope-Labeled Compounds

Antibodies

Natural
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