Anti-PL7 antibodies inhibit ThrRS, disrupting tRNA aminoacylation and triggering autoimmune responses. This antibody is linked to:
Immune dysregulation: ThrRS inhibition activates pro-inflammatory pathways (e.g., JNK), contributing to tissue damage in muscles and lungs .
Clinical associations: Strong correlation with ILD (≥90% of cases) and milder myositis compared to other antisynthetase antibodies .
ILD progression: Anti-PL7-associated ILD often presents as nonspecific interstitial pneumonia (NSIP) or organizing pneumonia (OP) .
Overlap syndromes: Co-occurrence with anti-Ro52 (63% of cases) and scleroderma-like symptoms .
Anti-PL7 detection relies on:
ELISA: Commercial kits (e.g., Human anti-PL7 ELISA) use a sandwich assay with HRP-conjugated antibodies (detection limit: 450 nm) .
Immunodot/Western blot: Confirms specificity using recombinant ThrRS antigens .
Clinical criteria: Requires ≥1 ASS symptom (e.g., ILD, myositis) and antibody positivity .
| Therapy | Response Rate | Source |
|---|---|---|
| Mycophenolate mofetil | 100% stability | Retrospective cohort |
| Cyclophosphamide | 75% improvement | Case study |
| Sildenafil (for PH) | Normalized NT-proBNP | Longitudinal follow-up |
KEGG: ath:AT3G55940
STRING: 3702.AT3G55940.1