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NPC1

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All listed services and products are For Research Use Only. Do Not use in any diagnostic or therapeutic applications.

Background

NPC intracellular cholesterol transporter 1 (NPC1) is a large multi-pass endolysosomal integral membrane protein encoded by NPC1 gene, functioning as the primary sensor and efflux transporter mediating cholesterol export from late endosomal and lysosomal compartments toward cytoplasmic organelles including endoplasmic reticulum and Golgi apparatus, acting synergistically with soluble luminal NPC2 cholesterol binding protein to execute sequential lipid transfer reactions. After lipoprotein particles undergo lysosomal proteolytic degradation, free cholesterol molecules are captured by NPC2 within acidic lysosomal lumens and delivered to the large extracellular N-terminal cholesterol binding pocket of membrane-embedded NPC1; conformational rearrangements across NPC1's multiple transmembrane helical segments then shuttle sterol substrates across lysosomal limiting membranes to exit into cytoplasmic lipid transport pathways. Under basal physiological lipid homeostasis, balanced NPC1-NPC2 coordinated transport maintains low lysosomal free cholesterol accumulation and supplies sterol substrates for cellular membrane biosynthesis and steroid hormone generation. Loss of functional NPC1 abolishes lysosomal cholesterol efflux capacity, triggering massive unesterified cholesterol and glycosphingolipid storage within endolysosomal compartments to drive progressive lysosomal dysfunction and multi-organ degenerative pathology characteristic of Niemann-Pick type C lysosomal storage disorder. Distinct from plasma membrane cholesterol transporters governing extracellular sterol uptake, NPC1 exclusively operates within acidic degradative organelles and bears unique multi-domain sterol sensing and transfer architecture that cannot be compensated by other lipid carrier proteins. Mild NPC1 expression fluctuations alter cellular cholesterol distribution profiles and impact lipid-dependent signaling cascades, while complete NPC1 functional deficiency leads to fatal progressive neurodegeneration and visceral organ lipid accumulation, establishing NPC1 as a core research target for endolysosomal lipid transport biology and lysosomal storage disorder therapeutic exploration.

Fig. 1 NPC1 mutant neuronal mechanism diagram for pathogenic phenotype analysis and drug efficacy evaluation. (OA Literature)Fig. 1 NPC1-D1005G mutation disturbs cholesterol balance in specific neuronal models.1

NPC1 Protein Function: Core Roles in Lysosomal Cholesterol Export, NPC2 Lipid Coordination and Sterol Homeostasis Control

The biological functions of NPC1 are focused on endolysosomal sterol sensing, NPC2-mediated cholesterol transfer and cytoplasmic lipid redistribution:

  • NPC2 Coupled Lipid Transport: Accepts cholesterol from luminal NPC2 to execute transmembrane efflux from lysosomes.
  • Multi-Domain Sterol Sensing: Intramembrane sterol-sensing domains monitor intralysosomal cholesterol levels to adjust transport activity.
  • Lysosomal Lipid Clearance: Prevents toxic unesterified cholesterol and glycosphingolipid accumulation within degradative organelles.
  • Cellular Sterol Supply: Delivers recycled cholesterol to ER and Golgi for membrane and steroid biosynthetic pathways.
  • Disease Relevance: Loss-of-function NPC1 mutations cause severe progressive Niemann-Pick type C lipid storage neurodegenerative disease.

NPC1 Protein Product

Creative Biolabs offers high-quality NPC1 proteins through optimized expression systems, including full-length multi-pass lysosomal transporter and isolated N-terminal cholesterol binding domain variants. These products retain native conformational characteristics and NPC2-coupled cholesterol transfer biological activity, suitable for lysosomal lipid transport interaction assays and lipid storage disorder regulatory compound screening. All NPC1 proteins undergo strict quality control to ensure consistent performance and reliable application across diverse research platforms.

NPC1 Membrane Protein Product

Not finding the membrane protein product you need? Contact us to start your one-stop custom service!

NPC1 Stable Cell Line Product

Creative Biolabs provides custom-engineered NPC1 stable cell lines, including wild-type and loss-of-function mutant expression control models. These cell lines are optimized for lysosomal cholesterol storage profiling and sterol efflux functional analysis. Each cell line undergoes stringent validation to ensure stable expression profiles and consistent functional performance in diverse experimental contexts.

NPC1 Stable Cell Line Product

Not finding the stable cell line product you need? Contact us to start your one-stop custom service!

NPC1 Recombinant Antibody Product

High-specificity recombinant antibodies targeting NPC1 are developed via advanced antibody engineering technologies, with no cross-reactivity with other lysosomal multi-pass lipid transporters. These antibodies are validated for late endosome/lysosome subcellular localization detection and neural tissue lipid transporter expression profiling, and can be matched with NPC2 detection reagents to analyze complete cholesterol transfer complex distribution in lipid storage cell models.

NPC1 Recombinant Antibody Product

Not finding the recombinant antibody product you need? Contact us to start your one-stop custom service!

Product Features

  • Native Cholesterol Transport Activity: Preserves intact NPC2 binding and sterol efflux capacity for lysosomal lipid homeostasis research.
  • Lysosomal Transporter Specificity: Eliminates non-specific cross-recognition of unrelated multi-pass lipid carrier proteins.
  • Lipid Storage Disease Compatibility: Optimized reagent series for Niemann-Pick C therapeutic and lipid trafficking modulator screening workflows.
  • Comprehensive Customization Support: Facilitates end-to-end development of customized proteins, antibodies and stable cell lines to address endolysosomal sterol transport research demands.

Custom NPC1 Research Services

Beyond catalog products, Creative Biolabs offers specialized custom services for NPC1 research:

  • Custom NPC1 Protein Production: Tailored expression of mutant and domain-truncated NPC1 constructs for NPC2 cholesterol binding transport analysis.
  • Custom Antibody Development: Generation of NPC1-specific antibodies for lysosomal membrane immunostaining in neural and visceral tissue samples.
  • Stable Cell Line Engineering: Construction of NPC1 mutant/wild-type cell models for lysosomal lipid storage and sterol efflux research.
  • Functional Assay Development: Custom design of lysosomal cholesterol accumulation and efflux detection workflows.

Frequently Asked Questions (FAQ)

  1. What is the primary function of NPC1?

    NPC1 is a multi-pass lysosomal transporter that collaborates with NPC2 to export free cholesterol from acidic endolysosomal compartments to cytoplasmic organelles.

  2. Why is NPC1 a significant research target?

    Pathogenic NPC1 variants block cholesterol efflux and trigger fatal Niemann-Pick type C lipid storage neurodegeneration, making it core lysosomal disorder research target.

  3. Are Creative Biolabs' NPC1 products suitable for clinical use?

    No, all NPC1 products and services are strictly for research use only, not intended for clinical diagnosis or treatment.

  4. What types of NPC1 products does Creative Biolabs offer?

    Offerings include full-length NPC1 lysosomal sterol transporters, target-specific detection antibodies and custom stable cell lines for lipid trafficking and storage disease research.

  5. How are NPC1 proteins validated for activity?

    NPC1 proteins are validated via NPC2 coupled cholesterol transmembrane transfer functional testing.

Reference
  1. Mitroi, Daniel N., et al. "NPC1 enables cholesterol mobilization during long‑term potentiation that can be restored in Niemann–Pick disease type C by CYP46A1 activation." The EMBO Reports 20.11 (2019): EMBR201948143. Under Open Access license CC BY 4.0, without modification. https://doi.org/10.15252/embr.201948143
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