Analytical Data
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Gene name
Niemann Pick C2/NPC2
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简介
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.
- Application
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Alternative Names
NPC intracellular cholesterol transporter 2; Niemann-Pick disease type C2 protein; HE1
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Species
Human
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Source
HEK293
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Tag
C-His
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
P61916-1
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Expression Region
E20-L151
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Protein Length
Full Length of Isoform-1 Mature Protein
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Molecular Weight
21-24 kDa & 27-35 kDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
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Protein Description
Niemann-Pick type C2 (NPC2) is a crucial protein involved in intracellular lipid transport, specifically the transport of cholesterol and other lipids within cells. Mutations in the NPC2 gene lead to Niemann-Pick disease, a rare, neurodegenerative disorder characterized by the accumulation of unesterified cholesterol and other lipids in lysosomes. This accumulation results in progressive neurodegeneration, organomegaly, and other debilitating symptoms. Research into NPC2 and its recombinant protein forms is vital for understanding the molecular mechanisms underlying the disease and for developing potential therapeutic strategies. Recombinant NPC2 protein studies provide insights into its structure, function, and interaction with lipid membranes, which are critical for elucidating how lipid transport is disrupted in Niemann-Pick disease. Furthermore, the availability of recombinant NPC2 allows for the exploration of gene therapy approaches aimed at restoring proper lipid metabolism in affected patients. Overall, NPC2 research not only enhances our understanding of a complex metabolic disorder but also holds promise for innovative treatments that could significantly improve the quality of life for individuals affected by Niemann-Pick disease.
