Search Result

Search Result

Results for "

Niemann-Pick disease type C

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Amino Acid Derivatives (1) Bacterial (1) Beta-lactamase (1) Endogenous Metabolite (1) Fluorescent Dye (1) HSP (3)
By Research Areas:	Cancer (1) Infection (2) Inflammation/Immunology (4) Metabolic Disease (5) Neurological Disease (6)

9

Inhibitors & Agonists

1

Natural
Products

3

Recombinant Proteins

Targets Recommended: Scavenger Receptor Class B type I (SR-BI） PCSK9

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

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 .

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 .

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.

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 .

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.

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.

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.

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.

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 .

Cat. No.	Product Name	Category	Target	Chemical Structure

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 .

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

Cat. No.	Compare	Product Name	Species	Source

	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.

	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.

	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.

Compare Products


Products	In-stock - + Add to Cart
Cat. No.
Species
Source
Tag
Accession
Gene ID
Molecular Weight
Purity
Endotoxin Level
Biological Activity
Appearance
Formulation
Storage & Stability
Shipping
Free Sample	Yes No
Size	* This product has been "discontinued". Optimized version of product available: / In-stock - + Add to Cart Get quote

Inquiry Online

Your information is safe with us. * Required Fields.

MCE Japan Authorized Agent ナミキ商事株式会社コスモ・バイオ株式会社
Salutation			Country or Region *
Applicant Name *			Organization Name *
Department *
Email Address *			Product Name *
Cat. No.			Requested quantity *
Phone Number *
Remarks

Inquiry Online

Inquiry Information

Product Name:
Cat. No.:
Quantity:
MCE Japan Authorized Agent:

MedChemExpress Magic Cece

MedChemExpress: Contact Us; About Us; Headquarters; Distributors; Statement on False Report; Careers

Customer Support: Technical Support; Service; Ordering Information; Easy Return; Shipping Rates & Policies; Intellectual Property Promise

Resources: Free Sample; Resources; Molarity Calculator; Dilution Calculator; Terms & Conditions; Reconstitution Calculator; Specific Activity Calculator

Subscribe to our E-newsletter

Name
Email *

	Sorry, but the email address you supplied was invalid.

Products are chemical reagents for research use only and are not intended for human use. We do not sell to patients.

Copyright © 2013-2024 MedChemExpress. All Rights Reserved.: Site Map Privacy Policy

Join with us