EXT2 Antibody
Description
Introduction to EXT2 Antibody
The EXT2 antibody targets the EXT2 protein, encoded by the EXT2 gene located on chromosome 11p . This gene is part of the EXT family (EXT1, EXT2, EXTL1-3), which synthesizes heparan sulfate (HS), a glycosaminoglycan essential for cell signaling, tissue repair, and embryonic development . Mutations in EXT2 cause Hereditary Multiple Exostoses (HME), an autosomal dominant disorder characterized by benign bone tumors . EXT2 antibodies are widely used to investigate HS biosynthesis, HME pathogenesis, and EXT2-associated autoimmune conditions like membranous nephropathy (MN) .
Research Applications of EXT2 Antibodies
EXT2 antibodies are utilized across diverse methodologies:
Key findings using these antibodies include:
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EXT1/EXT2 complex detection in 11.6% of PLA2R-negative MN cases, often linked to autoimmune markers like lupus .
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Co-localization studies confirming EXT1/EXT2 interaction in the Golgi, critical for HS chain elongation .
Hereditary Multiple Exostoses (HME)
EXT2 mutations account for ~35% of HME cases. Antibodies help identify pathogenic variants and study HS dysregulation in chondrocyte differentiation .
EXT1/EXT2-Associated Membranous Nephropathy (MN)
EXT2 antibodies have identified a novel MN subtype characterized by:
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Autoimmune associations: 80.7% of EXT1/EXT2-MN patients show autoimmune markers (e.g., antinuclear antibodies) .
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Demographics: Female predominance (4:1 ratio) and younger onset (mean age: 35.7 years) .
| Parameter | EXT1/EXT2-Positive MN (n=26) | EXT1/EXT2-Negative MN (n=198) |
|---|---|---|
| Autoimmune Markers | 80.7% | 29.2% |
| Lupus Diagnosis | 34.6% | 6.1% |
| GBM Staining (IgG) | 100% (2–3+/3) | 100% (1–3+/3) |
Data adapted from Ravindran et al. (2021) .
Future Directions
EXT2 antibodies are advancing research in:
Product Specs
Composition: 50% Glycerol, 0.01M PBS, pH 7.4
Target Background
Q&A
Here’s a structured collection of FAQs for researchers working with EXT2 antibodies, synthesized from peer-reviewed studies and technical guidelines:
How to resolve contradictions in EXT2 localization data across studies?
Advanced Analysis:
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Context-dependent localization: EXT2 primarily localizes to ER when expressed alone but shifts to Golgi when co-expressed with EXT1 . Ensure co-expression status is documented.
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Fixation artifacts: Compare methanol/acetone vs. paraformaldehyde fixation, as HS-protein complexes are sensitive to permeabilization .
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Cell-type variability: Test multiple models (e.g., HEK293 vs. sog9 cells) to assess tissue-specific trafficking .
What controls are critical for EXT2 antibody-based assays?
Experimental Design Framework:
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Negative controls:
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Positive controls:
How to optimize EXT2 detection in heparan sulfate (HS) biosynthesis studies?
Technical Guidelines:
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Sample preparation: Use 0.1% saponin for permeabilization to preserve Golgi integrity .
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Antibody dilution: Start with 1:200–1:500 (empirically determine using titration series).
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Cross-validation: Pair IF with HS disaccharide analysis via LC-MS to correlate EXT2 levels with HS sulfation patterns .
What advanced techniques leverage EXT2 antibodies in mechanistic studies?
Research Applications:
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Dynamic complex analysis:
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Functional assays:
How to interpret conflicting EXT2 expression data in cancer models?
Data Contradiction Resolution:
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Tumor heterogeneity: Distinguish between stem cells (low HS) vs. differentiated cells (high HS) using CD44/CD24 co-staining .
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Post-translational regulation: Assess EXT2 degradation via cycloheximide chase assays if mRNA/protein levels disagree .
