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MPZ

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

Background

The MPZ gene at 1q23.3 encodes myelin protein zero (P0), the most abundant protein of peripheral nerve myelin comprising over half of total myelin protein and a member of the immunoglobulin superfamily. MPZ is a single-pass type I transmembrane glycoprotein, with a large extracellular immunoglobulin-like domain, a single transmembrane helix, and a basic cytoplasmic tail that interacts with acidic lipids on the opposing membrane face. Its molecular weight is approximately 28 kDa and it is specifically expressed in myelinating Schwann cells of the peripheral nervous system, where it localizes to compact myelin. As the major adhesion molecule of peripheral myelin, P0 mediates homophilic interactions between adjacent myelin wraps via its extracellular Ig-like domain, forming homotetramers that drive myelin compaction essential for rapid nerve conduction. MPZ mutations cause autosomal dominant peripheral neuropathies including Charcot-Marie-Tooth disease type 1B (CMT1B), Dejerine-Sottas syndrome, and congenital hypomyelination neuropathy. Over 200 mutations have been documented, with mechanisms including ER retention and UPR activation, protein mistrafficking, and disrupted adhesion. MPZ mutations account for approximately 10% of demyelinating CMT cases. MPZ is an important target for studying myelin biology, peripheral neuropathy mechanisms, and Schwann cell function, providing tools for understanding myelin compaction and inherited peripheral neuropathies.

Fig.1 Schematic of the MPZ minigene. (OA Literature)Fig. 1 Schematic representation of the MPZ minigene construct.1

Function of MPZ Protein: A Major Adhesion Molecule in Peripheral Nerve Myelination and Compaction

The functional significance of MPZ spans multiple aspects of peripheral nerve biology:

  • Myelin compaction and adhesion: MPZ encodes P0, the most abundant peripheral myelin protein, which mediates homophilic adhesion between apposing myelin membranes via its extracellular Ig-like domain. P0 forms tetramers that interlock across the intraperiod line. Without functional P0, myelin fails to compact, impairing conduction.
  • Schwann cell differentiation and maintenance: P0 expression marks myelinating Schwann cells and guides Schwann cell wrapping around axons. Loss of P0 function leads to demyelination, axonal degeneration, and progressive neuropathy.
  • Genetic variants and disease mechanisms: Over 200 MPZ mutations cause hereditary neuropathies. Early-onset CMT1B mutations typically disrupt the Ig-domain fold, causing ER retention and UPR activation. Late-onset mutations often affect surface residues or the cytoplasmic domain, producing distinct phenotypes through altered adhesion or signaling.
  • Structure-function investigations: Structural studies of the MPZ extracellular domain have defined the Ig-fold and tetramerization interfaces, providing mechanistic insight into how different amino acid substitutions produce demyelinating versus axonal disease phenotypes.

MPZ Membrane Protein Product

Creative Biolabs offers a series of high-quality MPZ membrane protein products, suitable for structural and functional studies of peripheral myelin biology. Built on the independently developed MemDX™ recombinant membrane protein technology platform, MPZ proteins can be generated through various expression systems, including virus-like particles (VLP) and in vitro prokaryotic expression systems. These thoroughly characterized protein products, such as MemDX™ recombinant human MPZ membrane protein as virus-like particles and human MPZ proteins from diverse expression systems, can be used in ELISA, antibody screening and characterization, protein interaction assays, and functional investigations, providing researchers with reliable tools for analyzing the homophilic adhesion mechanism of P0 and the molecular basis of myelin compaction.

MPZ Protein Product

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

MPZ Stable Cell Line Product

Constructing a robust cell model is essential for investigating the molecular mechanisms of myelin compaction and MPZ-related neuropathy pathogenesis. As the major adhesion molecule mediating homophilic interaction between myelin membranes, the structural integrity of P0 directly determines myelin sheath compaction and axonal integrity. Genetic mutations may lead to substantial differences in adhesion properties and myelinating capacity. Creative Biolabs offers customized MPZ membrane protein stable transfection cell models, encompassing wild-type and disease-associated variant forms. These models express the target protein on the plasma membrane, and are suitable for studies on MPZ homophilic adhesion, analysis of variant-specific trafficking defects, and evaluation of ER stress responses, providing a reliable platform for exploring P0-mediated adhesion, the consequences of CMT-associated mutations, and Schwann cell myelination mechanisms. Our technology ensures cell membrane integrity and correct protein localization, facilitating your research.

MPZ Stable Cell Line Product

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

MPZ Recombinant Antibody Product

Creative Biolabs offers highly specific recombinant antibodies targeting MPZ, manufactured using advanced recombinant antibody technology. Compared to traditional antibodies, these recombinant antibodies deliver improved performance in specificity, sensitivity, and batch-to-batch consistency. They also support various modifications and labeling strategies. Our MPZ recombinant antibodies are suitable for a broad range of applications, including Western Blot, ELISA, flow cytometry, immunofluorescence, immunocytochemistry, immunohistochemistry, and immunoprecipitation, and can be used to examine MPZ expression levels in Schwann cells and peripheral nerve tissues, analyze its subcellular localization at the plasma membrane and within compact myelin, and conduct related investigations such as protein interaction studies and myelin compaction analysis. These antibodies provide dependable detection reagents for the structural characterization, functional investigation of MPZ, and the analysis of its role in peripheral nerve myelination and adhesion.

MPZ Recombinant Antibody Product

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

Product Features

  • Multiple expression platforms: A variety of platforms for recombinant protein production are available (such as virus-like particles and prokaryotic/eukaryotic expression systems), accommodating diverse research requirements.
  • Broad application compatibility: Products can be used in ELISA, antibody screening and characterization, protein interaction assays, Western Blot, immunofluorescence, and immunohistochemistry experiments.
  • Consistent batch performance: Stringent quality control measures maintain stability across product batches, ensuring dependable and reproducible experimental outcomes.
  • Tailored customization options: Customized services are available for specified natural variant forms, tag configurations, or expression platforms.

Custom MPZ Membrane Protein and Antibody Discovery Services

In addition to the standard products, Creative Biolabs also offers professional customized services for MPZ. MPZ has multiple documented genetic variants with functional differences, including CMT1B-associated mutations causing protein misfolding, mistrafficking, or adhesion defects. These variants present specific requirements in protein expression, trafficking analysis, and functional characterization. Leveraging our technology platform, we can assist you in completing the following customized projects:

  • Customized production of membrane proteins: Tailored expression (using multiple expression systems), purification, and molecular characterization for wild-type and variant forms.
  • Customized antibody development: From antigen design to antibody engineering (monoclonal, polyclonal, recombinant antibodies), supporting variant discrimination or recognition of shared epitopes.
  • Construction of stable cell model: Generate stable cell models expressing specific MPZ variants, for functional comparison studies and myelin compaction analysis.
  • Development of the functional detection system: Design and execute experimental protocols such as adhesion assays, protein interaction analysis, and functional evaluation of genetic variants.

Frequently Asked Questions (FAQ)

  1. What is the main function of the MPZ gene?

    MPZ encodes myelin protein zero (P0), the most abundant protein in peripheral nerve myelin, which functions as a homophilic adhesion molecule mediating compaction between adjacent myelin wraps. Its extracellular immunoglobulin-like domain forms tetramers that interlock across the intraperiod line, maintaining the multilamellar myelin structure essential for rapid saltatory conduction. P0 is specifically expressed in myelinating Schwann cells and is required for myelin sheath formation and long-term axonal integrity.

  2. Why is MPZ an important research target?

    MPZ is the gene responsible for Charcot-Marie-Tooth disease type 1B (CMT1B) and related peripheral neuropathies, with over 200 documented disease-causing mutations. Studying MPZ provides insight into the molecular mechanisms of myelin compaction, the structural determinants of homophilic adhesion, and the diverse pathomechanisms—including ER stress, protein mistrafficking, and adhesion defects—by which mutations cause peripheral nerve disease. MPZ is therefore a target of significant interest in myelin biology and inherited neuropathy research.

  3. Is the MPZ product of Creative Biolabs suitable for clinical applications?

    Not applicable. All MPZ products and services provided by Creative Biolabs are for research purposes only and cannot be used for any clinical diagnosis, prevention, treatment or cure of any disease.

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

    We offer a variety of product types, including recombinant MPZ membrane proteins, stable cell models expressing MPZ, high-quality recombinant MPZ antibodies, as well as comprehensive customized services.

Reference
  1. Maino, Anthony, et al. "Novel Dominant Splicing Variant in MPZ Associated With Unusual Charcot–Marie–Tooth Disease." Journal of the Peripheral Nervous System 30.4 (2025): e70085. Under Open Access license CC BY 4.0, cropped from the original figure. https://doi.org/10.1111/jns.70085
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