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Elastin (Structure (Primary Structure (Tropoelastin polypeptide (composed…

- - - - composed of
        
        Glycine
        
        Alanine
        
        Valine
        
        Lysine
        
        Proline
  - - - created by
        
        oxidative deamination by lysyl oxidase
  - - - glycoprotein microfibrils
- - - - mutations in fibrillin-1
    - - impaired structural integrity
  - - - mutations in alpha 1 antitrypsin
        
        leads to
        
        misfolded aggregates of AAT in liver
    - - unopposed neutrophil elastase action
        
        which leads to
        
        degraded alveolar elastin and pulmonary damage