Table 1.
Association of Defensins and LL‐37 with Various Diseases
| Respiratory Tract |
| Cystic fibrosis |
| ‐ reduced hBDs; elevated HNPs and LL‐37 associated with inflammation; inhibition of defensin activity by high ionic concentrations in respiratory lining fluid; degradation of defensins by proteases |
| Bronchiolitis obliterans and diffuse panbronchiolitis |
| ‐ elevated hBDs |
| ARDS and α1‐antitrypsin deficiency |
| ‐ elevated HNPs |
| Reactive airways disease |
| ‐ reduced hBD2 and LL‐37 in mouse model |
| Tobacco smoking |
| ‐ reduced hBD2 |
| Lung infections |
| ‐ Low LL‐37 associated with vitamin D deficiency increases risk for tuberculosis. |
| ‐ Defensins and LL‐37 contribute to host defense against other bacteria and respiratory viruses based on in vitro and mouse models. |
| ‐ Hyper‐IgE syndrome is associated with risk for lung and skin infections, in part, as a result of lack of production of antimicrobial peptides by bronchial epithelial cells or keratinocytes. |
| Gastrointestinal Tract |
| CD of ileum |
| ‐ reduced HD5 and HD6 as a result of impaired NOD2 and/or Wnt/Tcf‐4 signaling |
| CD of colon |
| ‐ reduced hBD2 and LL‐37 response; reduced hBD copy number |
| Ulcerative colitis |
| ‐ elevated hBD2 and LL‐37 |
| Helicobacter pylori infection of stomach |
| ‐ hBD2, hBD3, and LL‐37 participate in host response in vitro. |
| Bacterial dysentery |
| ‐ HD5 overexpression protects mice from Salmonella infection in the gut. |
| ‐ Mouse cryptdin expression correlates with inhibition of Shigella infection. |
| ‐ Shigella and enteropathogenic Escherichia coli interfere with hBD or LL‐37 expression; Salmonella alters LPS structure to avoid binding to antimicrobial peptides. |
| Oral Cavity |
| ‐ Morbus Kostmann syndrome is characterized by severe periodontitis associated with low levels of LL‐37 in neutrophils and saliva. |
| Genitourinary Tract |
| Bacterial vaginosis |
| ‐ reduced HNP and hBD levels in vaginal fluid |
| Neisseria gonorrhoeae urethritis and cervicitis |
| ‐ elevated HD and HNP levels in urethral and vaginal secretions; HD enhances HIV infectivity |
| HIV |
| ‐ reduced vaginal hBD levels |
| Urinary tract infection |
| ‐ mBD and CRAMP gene deletion in mice increase risk. |
| Skin |
| Atopic dermatitis |
| ‐ reduced hBD2 and LL‐37 levels in lesions |
| Hyper‐IgE syndrome |
| ‐ reduced levels of antimicrobial peptide production by keratinocytes associated with atopy and recurrent staphyloccal infections |
| Chronic skin ulcers |
| ‐ reduced antimicrobial peptide expression in ulcers |
| Psoriasis |
| ‐ increased hBD2 and LL‐37 in lesions; elevated hBD gene copy number |
| Acne rosacea |
| ‐ increased LL‐37 in lesions |