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Niemann-Pick disease

" in MedChemExpress (MCE) Product Catalog:

By Targets:	Amino Acid Derivatives (1) Bacterial (1) Beta-lactamase (1) Biochemical Assay Reagents (2) Drug Metabolite (1) Endogenous Metabolite (7) Fluorescent Dye (1) HSP (3) Isotope-Labeled Compounds (1) PKC (1) Reference Standards (1)
By Research Areas:	Cancer (2) Endocrinology (1) Infection (1) Inflammation/Immunology (5) Metabolic Disease (11) Neurological Disease (12)

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Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

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-113498

Sphingomyelin 1 Publications Verification	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-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-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.

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-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 .

HY-N15891

Glyco-3ß,5α,6ß -trihydroxycholanoic acid (NPCBA2)	Endogenous Metabolite	Neurological Disease
Glyco-3ß,5α,6ß -trihydroxycholanoic acid (NPCBA2) is a bile acid which accumulates preferentially in Niemann-Pick C (NPC) patients. Glyco-3ß,5α,6ß -trihydroxycholanoic acid is a biomarker for the early diagnosis of Niemann-Pick 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-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-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-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-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-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.

HY-153830S

LacCer (d18:1/16:0)-d3 C16 Lactosylceramide (d18:1/16:0)-d₃	Isotope-Labeled Compounds Endogenous Metabolite	Neurological Disease
LacCer (d18:1/16:0)-d₃ (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). 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-172546

Lyso-monosialoganglioside GM2 ammonium LysoGM2 ammonium	PKC	Metabolic Disease
Lyso-monosialoganglioside GM2 (LysoGM2) ammonium is a lysosphingolipid and protein kinase C inhibitor (IC₅₀: 50 μM). Lyso-monosialoganglioside GM2 ammonium can inhibit the binding of protein kinase C to Phorbol 12,13-dibutyrate (HY-18985). Lyso-monosialoganglioside GM2 ammonium is a specific biomarker for GM2 gangliosidosis. Lyso-monosialoganglioside GM2 ammonium can be used in the research of sphingolipidoses .

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 .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-113498

Sphingomyelin 1 Publications Verification	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 .

HY-59291

Levacetylleucine N-Acetyl-L-leucine	Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	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 Source classification Saccharides Monosaccharides	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 .

HY-N15891

Glyco-3ß,5α,6ß -trihydroxycholanoic acid (NPCBA2)	Lipid	Endogenous Metabolite
Glyco-3ß,5α,6ß -trihydroxycholanoic acid (NPCBA2) is a bile acid which accumulates preferentially in Niemann-Pick C (NPC) patients. Glyco-3ß,5α,6ß -trihydroxycholanoic acid is a biomarker for the early diagnosis of Niemann-Pick C disease .

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

Cat. No.	Compare	Product Name	Species	Source

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)-d₃ (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). 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 .

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

LacCer (d18:1/16:0)-d₃ (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). 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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