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NPHS2

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

Background

NPHS2 (podocin) is a lipid raft-associated stomatin family membrane protein encoded by the NPHS2 gene and highly enriched at the slit diaphragm of glomerular podocytes. Unlike widely expressed stomatin family members, podocin acts as a scaffold that interacts with slit diaphragm proteins such as nephrin and NEPH1, contributing to the structural integrity of the glomerular filtration barrier and limiting urinary loss of plasma proteins. Under physiological conditions, NPHS2 supports slit diaphragm organization and selective glomerular filtration. Biallelic pathogenic variants in NPHS2 can cause autosomal recessive steroid-resistant nephrotic syndrome (SRNS), often associated with focal segmental glomerulosclerosis (FSGS), with variable age of onset and disease severity. Other stomatin family members cannot fully compensate for the podocyte-specific function of NPHS2. Altered NPHS2 expression or function is also associated with podocyte injury and proteinuria, making it an important research target for podocyte biology and hereditary glomerular disease studies.

NPHS2 is anchored in the lipid raft region of podocyte podocyte membrane lipid bilayer, which plays a specific role in scaffold anchoring, forms a stable homodimer by relying on the conserved stomatin domain, physically bridges the nephrin-NEPH1 complex, and stabilizes the adhesion structure of the fissure membrane. Its strict glomerular tissue localization and lipid raft targeting characteristics form a clear functional differentiation with stomatin homologous protein distributed in pan-tissue, giving it exclusive ability to regulate renal filtration barrier. The cell connection stability mechanism mediated by NPHS2 can link podocyte cytoskeleton remodeling and glomerular filtration selectivity, and dynamically maintain the integrity of filtration barrier according to the development stage and injury degree of kidney. This molecule is involved in the differentiation and development of embryonic renal podocytes and the lifelong maintenance of adult glomerular filtration function. The expression defect or gene mutation of NPHS2 will completely destroy the diaphragm structure of hiatus and induce pathological proteinuria. To sum up, NPHS2 is a key research target for the study of podocyte membrane protein mechanism and the development of therapeutic targets for hereditary nephropathy.

Fig. 1 NPHS2-mediated glomerular slit diaphragm homeostasis and nephrotic syndrome pathological dual comparison schematic for podocyte and hereditary glomerulopathy research reagents. (OA Literature)Fig. 1 Graphical Abstract Schematic (a-b): Comparison of intact glomerular filtration barrier with normal NPHS2 podocin expression and defective slit diaphragm induced by NPHS2 deficiency leading to podocyte collapse and massive proteinuria.1

NPHS2 Protein Function: Core Roles in Slit Diaphragm Scaffolding and Renal Filtration Tuning

The biological functions of NPHS2 are fully focused on glomerular slit diaphragm complex assembly and renal filtration barrier protection:

  • Lipid Raft Scaffolding: Forms stable podocin homodimers to crosslink nephrin and NEPH1 slit diaphragm junction proteins.
  • Filtration Barrier Maintenance: Secures intact glomerular filter structure to block circulating plasma protein leakage.
  • Podocyte Homeostasis: Preserves complete foot process morphology under healthy resting renal conditions.
  • Glomerular Injury Modulation: Downregulated expression accelerates podocyte damage and pathological proteinuria.
  • Disease Relevance: Pathogenic biallelic NPHS mutations trigger congenital steroid-resistant nephrotic syndrome.

NPHS2 Protein Product

Creative Biolabs offers high-quality NPHS2 proteins through optimized eukaryotic expression systems, including full-length podocin transmembrane protein and isolated stomatin scaffold domain variants. These products retain native slit diaphragm complex binding activity, suitable for glomerular barrier and nephropathy inhibitor screening assays. All NPHS2 proteins undergo strict quality control to ensure consistent performance and reliable application across diverse renal research platforms.

NPHS2 Protein Product

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

NPHS2 Stable Cell Line Product

Creative Biolabs provides custom-engineered NPHS2 stable cell lines, including wild-type/mutant overexpression and blank control models. These cell lines are optimized for podocyte scaffold protein profiling and filtration barrier functional analysis. Each cell line undergoes stringent validation to ensure stable expression profiles during cell differentiation culture, and can be widely applied to glomerular injury compound screening experiments.

NPHS2 Stable Cell Line Product

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

NPHS2 Recombinant Antibody Product

High-specificity recombinant antibodies targeting NPHS2 are developed via advanced antibody engineering technologies, with no cross-reactivity with other stomatin family proteins. These antibodies are validated for glomerular podocyte foot process immunostaining, and can be combined with nephrin detection reagents to analyze slit diaphragm complexes in renal tissue models.

NPHS2 Recombinant Antibody Product

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

Product Features

  • Native Slit Diaphragm Scaffold Activity: Preserves intact nephrin binding capacity for glomerular research.
  • NPHS2 Specificity: Eliminates non-specific cross-recognition of ubiquitous stomatin paralogs.
  • Nephrology Compatibility: Optimized reagent series for hereditary nephrosis and glomerular injury screening workflows.
  • Comprehensive Customization Support: Facilitates end-to-end development of customized proteins, antibodies and stable cell lines to address podocyte research demands.

Custom NPHS2 Research Services

Beyond catalog products, Creative Biolabs offers specialized custom services for NPHS2 research:

  • Custom NPHS2 Protein Production: Tailored expression of mutant and tagged NPHS2 constructs for slit diaphragm binding analysis.
  • Custom Antibody Development: Generation of NPHS2-specific antibodies for glomerular podocyte immunostaining.
  • Stable Cell Line Engineering: Construction of NPHS2-modified podocyte cell models for barrier research.
  • Functional Assay Development: Custom design of glomerular protein leakage detection workflows.

Frequently Asked Questions (FAQ)

  1. What is the primary function of NPHS2?

    NPHS2 (podocin) is a podocyte stomatin scaffold protein that assembles slit diaphragm complexes to maintain the glomerular filtration barrier.

  2. Why is NPHS2 a significant research target?

    Recessive NPHS2 mutations are the leading cause of childhood steroid-resistant nephrotic syndrome, core marker for glomerular damage.

  3. Are Creative Biolabs' NPHS2 products suitable for clinical use?

    No, all NPHS2 products and services are strictly for research use only, not intended for clinical renal diagnosis or treatment.

  4. What types of NPHS2 products does Creative Biolabs offer?

    Offerings include full-length NPHS2 podocin proteins, isoform-specific detection antibodies and custom stable cell lines for glomerular research.

  5. How are NPHS2 proteins validated for activity?

    NPHS2 proteins are validated via nephrin co-binding and slit diaphragm stabilization functional testing.

Reference
  1. Lee, Jun Xin, Yan Jin Tan, and Noor Akmal Shareela Ismail. "NPHS mutations in pediatric patients with congenital and steroid-resistant nephrotic syndrome." International Journal of Molecular Sciences 25.22 (2024): 12275. Under Open Access license CC BY 4.0, without modification. https://doi.org/10.3390/ijms252212275
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