Table 1.
Autoantibodies associated with Cogan's syndrome.
| Autoantibody | Antigen location | Antigen function | References | |
|---|---|---|---|---|
| Human studies: number of CS patients/study design/country of publication | Animal models | |||
| Anti-HSP-70 | Not specific | Protein folding and ubiquitin-mediated degradation; protection from cell stress | 38/case control/Italy [27] | — |
| 14/case control/Italy [26] | ||||
| 8/case control/USA [71] | ||||
|
| ||||
| ANCA | Neutrophils | Proteolysis (PR3) | 5/case reports/France [72]; Japan [32, 73]; USA [30]; the Netherlands [33] | — |
| Production of oxidative free radicals (MPO) | ||||
|
| ||||
| Anti-Cogan peptide | Endothelial cells in the inner ear, lymphocytes, and kidney | Contact inhibition of cell growth; homolog with laminin, connexin 26, SSA/Ro, reovirus III major core protein lambda1, and cell density-enhanced protein tyrosine phosphatase-1 (DEP-1/CD148) | 8/case control/Italy [24] | Rabbits and mice [24] |
HSP-70: heat shock protein-70; CS: Cogan's syndrome; ANCA: anti-neutrophil cytoplasmic antibody; PR3: proteinase-3; MPO: myeloperoxidase.