20 Results for "

Niemann-Pick type C disease

" in MedChemExpress (MCE) Product Catalog:
Products (20)

20 Results for "Niemann-Pick type C disease" in MCE Product Catalog:

7
7 Publications Verification
Cat. No.: HY-106443A
CAS No.: 289893-26-1
Purity:  99.96%
Synonyms: BRX-220
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.
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7
7 Publications Verification
Cat. No.: HY-106443
CAS No.: 289893-25-0
Purity:  99.74%
Synonyms: BRX-220 free base
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 .
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7
7 Publications Verification
Cat. No.: HY-106443B
CAS No.: 368860-21-3
Purity:  99.76%
Synonyms: BRX-220 citrate
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.
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2
2 Cited Publications
Cat. No.: HY-116815
CAS No.: 501104-16-1
Purity:  99.75%
Research Areas:  

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 .
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1
1 Cited Publications
Cat. No.: HY-100353
CAS No.: 74713-60-3
Purity:  99.82%
Synonyms: N-Lauroyl-D-erythro-sphingosine; N-Laurylsphingosine
Target:  

Endogenous Metabolite

Research Areas:  

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 .
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Cat. No.: HY-59291
CAS No.: 1188-21-2
Synonyms: N-Acetyl-L-leucine
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 .
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Cat. No.: HY-153830
CAS No.: 4201-62-1
Synonyms: C16 Lactosylceramide (d18:1/16:0)
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 .
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Cat. No.: HY-W754473
CAS No.: 2517555-53-0
Category:  

Lipids

Lyso-SM-509, a sphingomyelin, is a biomarker for Niemann-Pick disease Type C .
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Cat. No.: HY-131682
CAS No.: 201731-19-3
Purity:  ≥99.0%
Synonyms: 3-Hexanoyl-NBD-cholesterol
Target:  

Fluorescent Dye

Research Areas:  

Others

3-C6-NBD-cholesterol is a fluorescent analog of Chol that can be used to measure the kinetics of membrane and intracellular trafficking .
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Cat. No.: HY-153830S
CAS No.: 2097249-92-6
Synonyms: C16 Lactosylceramide (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 .
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Cat. No.: HY-P74695
Purity:  ≥ 95%, as determined by reducing SDS-PAGE.
Synonyms: NPC1; Truncated Niemann-Pick C1; NPC Intracellular Cholesterol Transporter 1; Niemann-Pick C1; Niemann-Pick C1 Protein; POGZ; SLC65A1; NPC; Niemann-Pick disease, type C1
Species:  
Human
Source:  
HEK293
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Cat. No.: HY-100353R
CAS No.: 74713-60-3
Synonyms: N-Lauroyl-D-erythro-sphingosine (Standard); N-Laurylsphingosine (Standard)
Research Areas:  

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 .
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Cat. No.: HY-P810362
Synonyms: Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1_HUMAN

Host:  

Rabbit

Application:  

WB, ICC/IF, IF-Tissue, IHC-P, FC

Reactivity:  

Human, Mouse, Rat

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Cat. No.: HY-P74692
Purity:  ≥ 95%, as determined by reducing SDS-PAGE.
Synonyms: NPC2; Epididymal Protein 1; NPC Intracellular Cholesterol Transporter 2; NP-C2; HE1; Epididymis Secretory Sperm Binding Protein; EDDM1; Tissue-Specific Secretory Protein; Niemann-Pick disease type C2 Protein; Niemann-Pick disease, type C2; Human Epididymi
Species:  
Rat
Source:  
HEK293
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Cat. No.: HY-P74693
Purity:  ≥ 95%, as determined by reducing SDS-PAGE.
Synonyms: NPC2; Epididymal Protein 1; NPC Intracellular Cholesterol Transporter 2; NP-C2; HE1; Epididymis Secretory Sperm Binding Protein; EDDM1; Tissue-Specific Secretory Protein; Niemann-Pick disease type C2 Protein; Niemann-Pick disease, type C2; Human Epididymi
Species:  
Mouse
Source:  
HEK293
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Cat. No.: HY-P74694
Purity:  ≥ 95%, as determined by Bis-Tris PAGE.
Synonyms: NPC2; Epididymal Protein 1; NPC Intracellular Cholesterol Transporter 2; NP-C2; HE1; Epididymis Secretory Sperm Binding Protein; EDDM1; Tissue-Specific Secretory Protein; Niemann-Pick disease type C2 Protein; Niemann-Pick disease, type C2; Human Epididymi
Species:  
Human
Source:  
HEK293
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Cat. No.: HY-182938
CAS No.: 1111050-17-9
Target:  

Autophagy

Research Areas:  

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 .
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Cat. No.: HY-W1120833
m-PEG26-NHS ester is a PEG derivative. m-PEG26-NHS ester can be used for the study of Niemann-Pick type C1 disease .
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Cat. No.: HY-W414824
CAS No.: 1246298-66-7
Target:  

Endogenous Metabolite

Research Areas:  

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.
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Cat. No.: HY-106443AR
CAS No.: 289893-26-1
Synonyms: BRX-220 (Standard)
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.
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