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Niemann-Pick type C disease

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Amino Acid Derivatives (1) Autophagy (1) Bacterial (1) Beta-lactamase (1) Biochemical Assay Reagents (2) Endogenous Metabolite (4) Fluorescent Dye (1) HSP (4) Isotope-Labeled Compounds (1) Reference Standards (2)
By Research Areas:	Cancer (2) Infection (1) Inflammation/Immunology (5) Metabolic Disease (12) Neurological Disease (10)

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Recombinant Proteins

Isotope-Labeled Compounds

Antibodies

Targets Recommended: C-type Lectin-like Receptors (CTLRs) PCSK9 Scavenger Receptor Class B type I (SR-BI）

Art. -Nr.	Produktname	Target	Forschungsgebiete	Chemical Structure

HY-106443A

Arimoclomol maleate 5 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 5 Publications Verification 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. Arimoclomol can cross the blood-brain barrier and can be used in research on Niemann-Pick disease type C .

HY-59291

Levacetylleucine N-Acetyl-L-leucine	Amino Acid Derivatives	Neurological Disease Metabolic Disease Inflammation/Immunology
Levacetylleucine (N-acetyl-L-leucine), an orally bioavailable and brain-penetrant compound, 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-116815

Lalistat 1 2 Publications Verification	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-106443B

Arimoclomol citrate 5 Publications Verification 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-153830

LacCer (d18:1/16:0) C16 Lactosylceramide (d18:1/16:0)	Endogenous Metabolite	Neurological Disease Metabolic Disease
LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0)) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). The levels of LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. In a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder), the expression of LacCer (d18:1/16:0) is also upregulated. LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .

HY-100353

C12-Ceramide N-Lauroyl-D-erythro-sphingosine; N-Laurylsphingosine	Endogenous Metabolite	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-W754473

Lyso-SM-509	Biochemical Assay Reagents	Metabolic Disease
Lyso-SM-509, a sphingomyelin, is a biomarker for Niemann-Pick disease Type C .

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 .

HY-153830S

LacCer (d18:1/16:0)-d3 C16 Lactosylceramide (d18:1/16:0)-d₃	Isotope-Labeled Compounds Endogenous Metabolite	Neurological Disease Metabolic Disease
LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0))-d₃ is the deuterium labeled LacCer (d18:1/16:0) (HY-153830). LacCer (d18:1/16:0) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. LacCer (d18:1/16:0) is also upregulated in a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder). LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .

HY-100353R

C12-Ceramide (Standard) N-Lauroyl-D-erythro-sphingosine (Standard); N-Laurylsphingosine (Standard)	Reference Standards Others	Metabolic Disease Cancer
C12-Ceramide (Standard) is the analytical standard of C12-Ceramide. This product is intended for research and analytical applications. 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-182938

Autophagy activator-2	Autophagy	Metabolic Disease
Autophagy activator-2 is a potent autophagy activator with EC₅₀ values of 14.33 μM. Autophagy activator-2 acts as a proteostasis modulator and small-molecule chaperone that upregulates I1061T mutant NPC1 expression and facilitates cholesterol efflux. Autophagy activator-2 reduces lysosomal hydrolase levels. Autophagy activator-2 can be used for the study of Niemann-Pick Type C disease .

HY-W1120833

m-PEG26-NHS ester	Biochemical Assay Reagents	Neurological Disease Metabolic Disease
m-PEG26-NHS ester is a PEG derivative. m-PEG26-NHS ester can be used for the study of Niemann-Pick type C1 disease .

HY-W414824

3β,7β-Dihydroxy-5-cholestenoic acid	Endogenous Metabolite	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-106443AR

Arimoclomol maleate (Standard) BRX-220 (Standard)	Reference Standards HSP	Neurological Disease Metabolic Disease Inflammation/Immunology
Arimoclomol maleate (Standard) is the analytical standard of Arimoclomol maleate (HY-106443A). This product is intended for research and analytical applications. 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.

Art. -Nr.	Produktname	Category	Target	Chemical Structure

HY-59291

Levacetylleucine N-Acetyl-L-leucine	Classification of Application Fields Ketones, Aldehydes, Acids Metabolic Disease Endogenous metabolite Disease Research Fields Source Classification	Amino Acid Derivatives
Levacetylleucine (N-acetyl-L-leucine), an orally bioavailable and brain-penetrant compound, 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-153830

LacCer (d18:1/16:0) C16 Lactosylceramide (d18:1/16:0)	Microorganisms Saccharides Monosaccharides Source Classification	Endogenous Metabolite
LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0)) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). The levels of LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. In a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder), the expression of LacCer (d18:1/16:0) is also upregulated. LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .

HY-W754473

Lyso-SM-509	Lipid	Biochemical Assay Reagents
Lyso-SM-509, a sphingomyelin, is a biomarker for Niemann-Pick disease Type C .

Species:	Human (1) Mouse (1) Rat (1)
Tag:	His (3)
Source:	HEK293 (3)

Art. -Nr.	Compare	Produktname	Species	Source	Image

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.

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-153830S

LacCer (d18:1/16:0)-d3
LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0))-d₃ is the deuterium labeled LacCer (d18:1/16:0) (HY-153830). LacCer (d18:1/16:0) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. LacCer (d18:1/16:0) is also upregulated in a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder). LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .

LacCer (d18:1/16:0)-d3

LacCer (d18:1/16:0) (C16 Lactosylceramide (d18:1/16:0))-d₃ is the deuterium labeled LacCer (d18:1/16:0) (HY-153830). LacCer (d18:1/16:0) is an endogenous bioactive sphingolipid that can form membrane microdomains with Lyn kinase and the αi subunits of inhibitory G protein-coupled receptors (GPCRs). LacCer (d18:1/16:0) are elevated in the plasma of insulin-resistant cattle. LacCer (d18:1/16:0) is also upregulated in a mouse model of Niemann-Pick type C1 disease (a neurodegenerative cholesterol-sphingolipid lysosomal storage disorder). LacCer (d18:1/16:0) can be used in research on metabolic diseases and neurodegenerative diseases .

Art. -Nr.	Compare	Produktname	Application	Reactivity	Image

HY-P810362

	Niemann Pick C1 Like 1 Antibody (YA9684) Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1_HUMAN	WB, ICC/IF, IF-Tissue, IHC-P, FC	Human, Mouse, Rat
	Niemann Pick C1 Like 1 Antibody (YA9684) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to Niemann Pick C1 Like 1.

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