CFTR Specific Neutra™ Antibody Products

Are extended drug development timelines or challenges in validating CFTR functionality hindering your cystic fibrosis research? Creative Biolabs' CFTR specific Neutra™ antibody products leverage advanced recombinant protein engineering and high-throughput validation platforms to accelerate therapeutic discovery, enabling precise detection, functional modulation, and robust quantification of CFTR for transformative outcomes.

Introduction to CFTR

The cystic fibrosis transmembrane conductance regulator (CFTR) is a critical chloride channel regulating ion and water homeostasis across epithelial tissues. Mutations in CFTR disrupt fluid balance, leading to severe pathologies such as cystic fibrosis (CF), the most common autosomal recessive disorder in Caucasian populations.

Basic Characteristics

CFTR, encoded on chromosome 7 (7q31.2), comprises 1,480 amino acids and belongs to the ATP-binding cassette (ABC) transporter superfamily. Unlike typical ABC transporters, CFTR primarily functions as a cyclic AMP-dependent anion channel. Its expression spans epithelial cells in the lungs, pancreas, and intestines, where it modulates mucus viscosity and antimicrobial activity.

Structural Complexity

The protein's architecture includes two membrane-spanning domains (MSD1 and MSD2), two nucleotide-binding domains (NBD1 and NBD2), and a regulatory (R) domain. Channel gating requires phosphorylation of the R domain by protein kinase A, followed by ATP hydrolysis at the NBDs. Cryo-EM studies reveal that disease-causing mutations (e.g., ΔF508) destabilize NBD1-MSD1 interactions, impairing folding and trafficking—a key focus for rescue therapeutics.

Fig.1 CFTR schematic structure.^1,3

Signaling Interplay

CFTR intersects with pathways such as:

• Wnt/β-catenin signaling: CFTR dysfunction elevates ENaC activity, exacerbating airway dehydration.
• NF-κB signaling: Persistent inflammation in CF airways links to defective CFTR-mediated chloride secretion and unchecked pro-inflammatory cytokine production.
• ER stress pathways: Misfolded CFTR triggers unfolded protein response (UPR), driving cellular apoptosis in pancreatic ducts.

Disease Relevance

Over 2,000 CFTR mutations are documented, with Class II mutations (e.g., ΔF508) causing misfolding and premature degradation, accounting for 85% of CF cases. Beyond CF, CFTR dysregulation associates with chronic pancreatitis, bronchiectasis, and male infertility.

Fig.2 CFTR endoplasmic reticulum quality control.^2,3

Applications of Anti-CFTR Neutralizing Antibodies in Clinical Research

• Diagnostic Biomarker Development

Anti-CFTR antibodies enable early detection of CFTR dysfunction in neonatal screens or sweat chloride tests, improving diagnostic accuracy for atypical CF presentations.

• Therapeutic Candidate Validation

Antibodies serve as critical tools to evaluate CFTR correctors and potentiators in restoring channel activity. Neutralizing formats help identify off-target effects in combo therapies.

• Precision Gene Therapy

AAV-mediated CFTR delivery efficiency is assessed via antibody-based detection of transgene expression in airway epithelia, guiding dose optimization for clinical trials.

• Patient Stratification

Quantifying CFTR levels in nasal epithelial cells using IHC predicts individual responsiveness to CFTR modulators, enabling personalized treatment protocols.

Our Anti-CFTR Antibodies

Our anti-CFTR antibodies empower researchers to:

- Quantify CFTR expression: Validate protein levels in patient-derived organoids or gene-edited cell lines via ELISA or Western blot.

- Monitor trafficking efficiency: Use immunofluorescence to track ΔF508-CFTR rescue by correctors in real time.

- Modulate channel activity: Neutralizing antibodies block aberrant CFTR Cl^⁻ conductance in in vitro assays, supporting high-throughput drug screening.

- Characterize post-translational modifications: Detect phosphorylation-dependent activation of the R domain, critical for assessing therapeutic efficacy.

Validated across multiple platforms (IHC, flow cytometry), these antibodies exhibit <5% cross-reactivity with other ABC transporters, ensuring unparalleled specificity.

Creative Biolabs offers CFTR-specific Neutra™ antibody products, combining rigorous validation, scalability, and unmatched sensitivity to drive breakthroughs in cystic fibrosis research.

Contact our scientific team today to discuss your project requirements and request tailored solutions.

REFERENCES

1. Lopes-Pacheco, Miquéias. "CFTR modulators: shedding light on precision medicine for cystic fibrosis." Frontiers in pharmacology 7 (2016): 275. https://doi.org/10.3389/fphar.2016.00275
2. Fukuda, Ryosuke, and Tsukasa Okiyoneda. "Cystic fibrosis transmembrane conductance regulator (CFTR) ubiquitylation as a novel pharmaceutical target for cystic fibrosis." Pharmaceuticals 13.4 (2020): 75. https://doi.org/10.3390/ph13040075
3. Distributed under Open Access license CC BY 4.0, without modification.