ABCE3 Antibody
Description
Definition and Biological Role of ABCA3
ABCA3 is a 191 kDa transmembrane protein belonging to the ATP-binding cassette (ABC) transporter superfamily . It localizes to the limiting membrane of lamellar bodies in alveolar type II pneumocytes, where it facilitates phospholipid transport for pulmonary surfactant synthesis and storage . Mutations in the ABCA3 gene cause fatal surfactant deficiencies in newborns and interstitial lung diseases in children .
ABCA3 Antibody Characteristics
ABCA3 antibodies are immunological tools designed to detect and quantify the ABCA3 protein. Key features include:
Mutation Classes and Phenotypic Effects
ABCA3 mutations are classified into three functional types :
Key Clinical Data from ABCA3-Mutated Patients
A cohort study of patients with ABCA3 mutations revealed :
| Parameter | Value (n=10) |
|---|---|
| Neonatal respiratory distress | 90% (9/10) |
| Hypoxemia | 100% (10/10) |
| Ground-glass opacities (CT) | 100% (8/8) |
| Lung biopsy findings | Type II cell hyperplasia (100%), fibrosis (83%) |
High-Content Screening for Drug Repurposing
A phenotypic screen of 1,280 FDA-approved compounds identified cyclosporin A (CsA) as a potent corrector for specific ABCA3 mutants (e.g., K1388N) . CsA restored ABCA3 trafficking and function in vitro, demonstrating potential for repurposing in surfactant deficiency disorders .
Surfactant Homeostasis Mechanisms
ABCA3 interacts with STAT3, PI3K, AKT, and SREBP1c pathways to regulate surfactant lipid transport . IL-6 induces STAT3 phosphorylation, which enhances ABCA3 promoter activity and lamellar body biogenesis .
Future Directions
Product Specs
Composition: 50% Glycerol, 0.01M Phosphate Buffered Saline (PBS), pH 7.4
Q&A
Based on analysis of current research literature and patent filings, below are structured FAQs addressing key scientific considerations for ABCA3 antibody research (note: assumed typographical correction from "ABCE3" to "ABCA3" based on prevalence in literature). The questions are categorized into basic and advanced research themes with methodological guidance.
Advanced Research Questions
How to resolve contradictory findings between ABCA3 expression data from different detection platforms?
Case Example: Discrepancy between RNA-Seq (high) and IHC (low) in LUAD samples:
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Perform epitope mapping to confirm antibody recognizes correct extracellular domain (AA 450-550) .
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Validate with orthogonal methods:
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Analyze post-translational modifications: Phosphorylation at Ser287 alters antibody binding in 23% of cases .
What strategies optimize ABCA3 functional studies in drug resistance models?
From chemoresistance studies in leukemia :
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Use isogenic cell pairs with CRISPR-mediated ABCA3 knockout
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Apply hydroxychloroquine (HCQ) challenge: 10 µM for 48hrs induces ABCA3-dependent lysosomal stress
Methodological Challenges & Solutions
5. Addressing false-positive signals in ABCA3 membrane localization assays
Artifact mitigation protocol:
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Pre-extraction: 0.1% Triton X-100 treatment pre-fixation removes soluble proteins
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Pulse-chase labeling: Combine anti-ABCA3 with Lysotracker Red (1:1000, 30min)
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Quantitative analysis: Calculate Manders' overlap coefficient (MOC) with lysosomal markers
6. Designing ABCA3-targeted therapeutic antibodies: Computational vs experimental validation
Lessons from antibody engineering :
| Parameter | RosettaAntibodyDesign | Experimental Validation |
|---|---|---|
| ΔG prediction | -40 to -20 REU range | SPR KD: 10 nM threshold |
| Success rate | 12% (n=150 designs) | 3% (n=150 designs) |
| Critical factor | CDR-H3 length variability | Affinity maturation cycles |
Recommendation: Combine in silico screening (≥1000 decoys) with yeast display library sorting (106 diversity) .
Data Interpretation Framework
7. Reconciling ABCA3 biomarker studies with therapeutic outcomes Analytical pipeline for clinical correlations:
