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Results for "

Niemann-Pick disease

" in MedChemExpress (MCE) Product Catalog:

11

Inhibitors & Agonists

2

Natural
Products

3

Recombinant Proteins

Cat. No. Product Name Target Research Areas Chemical Structure
  • 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 .
    Sphingomyelin
  • 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 .
    Levacetylleucine
  • 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.
    Arimoclomol maleate
  • 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 .
    C12-Ceramide
  • 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 .
    Lalistat 1
  • HY-W778830
    Bodipy C12-ceramide

    		Drug Metabolite Neurological Disease
    Bodipy C12-ceramide (B12Cer) is a fluorescently tagged form of C12-Ceramide (HY-100353) that displays excitation/emission maxima of 505/540 nm, respectively. Bodipy C12-ceramide is formed when acid sphingomyelinase hydrolyzes BODIPY-C12 sphingomyelin in vitro and has been used to quantify acid sphingomyelinase activity in plasma with Niemann-Pick disease .
    Bodipy C12-ceramide
  • 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.
    3β,7β-Dihydroxy-5-cholestenoic acid
  • 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.
    Arimoclomol
  • 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.
    Arimoclomol citrate
  • HY-145549
    C20 Sphingomyelin (d18:1/20:0)

    N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine; SM(d18:1/20:0)

    		 Endogenous Metabolite Endocrinology
    C20 Sphingomyelin (d18:1/20:0) (N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine; SM(d18:1/20:0)) is a naturally occurring sphingolipid. C20 Sphingomyelin (d18:1/20:0) levels are upregulated in the hippocampus of streptozotocin (HY-13753)-induced diabetic rats and in human plasma, positively correlating with insulin resistance in obese humans. C20 Sphingomyelin (d18:1/20:0) is also upregulated in the liver of a mouse model of Niemann-Pick C1 disease, a neurodegenerative cholesterol sphingolipid lysosomal storage disorder.
    C20 Sphingomyelin (d18:1/20:0)
  • 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 .
    3-C6-NBD-cholesterol

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