Alpha-1 antitrypsin deficiency alleles and severe cystic fibrosis lung disease

Thorax

Volumn 53, Issue 12, 1998, Pages 1022-1024

  Mahadeva, R ^a   Stewart, S ^b   Bilton, D ^b   Lomas, D A ^a  

^a UNIVERSITY OF CAMBRIDGE   (United Kingdom)

^b PAPWORTH HOSPITAL   (United Kingdom)

Author keywords

Bronchiectasis; Cystic fibrosis; Serpins; 1 antitrypsin; 1 proteinase inhibitor

Indexed keywords

ALPHA 1 ANTITRYPSIN; DNA; FORMALDEHYDE; PROTEINASE INHIBITOR; RESTRICTION ENDONUCLEASE;

ALLELE; ALPHA 1 ANTITRYPSIN DEFICIENCY; ARTICLE; CHILD; CYSTIC FIBROSIS; DISEASE SEVERITY; GENE AMPLIFICATION; HUMAN; LUNG FUNCTION; LUNG PARENCHYMA; LUNG TRANSPLANTATION; MAJOR CLINICAL STUDY; PREVALENCE; PRIORITY JOURNAL;

EID: 0031704170     PISSN: 00406376     EISSN: None     Source Type: Journal    
DOI: 10.1136/thx.53.12.1022     Document Type: Article

References (21)

1. Suter S, Schaad UB, Tegner K, et al. Levels of free granulocyte elastase in bronchial secretions from patients with cystic fibrosis: effect of antimicrobial treatment against Pseudomonas aeruginosa. J Infect Dis 1986;153:902-9.
2. Goldstein W, Döring G. Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis. Am Rev Respir Dis 1986;134:49-56.
3. Cook PJL. Genetic aspects of the Pi system. Postgrad Med J 1974;50:362-4.
4. 1-antitrypsin deficiency. Acta Med Scand 1965;Suppl 432:1-85.
5. 1-Antitrypsin deficiency: evaluation of bronchiectasis with CT. Radiology 1996;199:137-41.
6. 1-antitrypsin in Puerto Rican children with asthma. Chest 1993;103:812-5.
7. Geddes DM, Webley M, Brewerton DA, et al. Alpha-1 antitrypsin phenotypes in fibrosing alveolitis and rheumatoid arthritis. Lancet 1977;ii:1049-51.
8. Hubbard R, Baoku Y, Kalsheker N, et al. Alpha-1 antitrypsin phenotypes in patients with cryptogenic fibrosing alveolitis: a case-control study. Eur Respir J 1997;10: 2881-3.
9. 1-antitrypsin deficiency alleles, the Taq-I G→A allele and cystic fibrosis lung disease. Eur Respir J 1998;11:873-9.
10. 1-antitrypsin in patients with cystic fibrosis, homozygous and heterozygous for deltaF508. Pediatr Pulmon 1994;18:3-7.
11. 1-antitrypsin gene. Clin Chem 1992;38:2100-7.
12. Starkey PM. Elastase and cathepsin G: the serine proteinases of human neutrophil leucocytes and spleen. In: Barrett AJ, ed. Proteinases in mammalian cells and tissues. Amsterdam: North-Holland, 1977: 57-87.
13. Smallman LA, Hill SL, Stockley RA. Reduction of ciliary beat frequency in vitro by sputum from patients with bronchiectasis: a serine proteinase effect. Thorax 1984;39: 663-7.
14. Sommerhoff CP, Nadel JA, Basbaum CB, et al. Neutrophil elastase and cathepsin G stimulate secretion from cultured bovine airway gland serous cells. J Clin Invest 1990;85:682-9.
15. Fick RB, Naegel GP, Squier SU, et al. Proteins of the cystic fibrosis respiratory tract: fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis. J Clin Invest 1984;74:236-8.
16. Suter S, Schaad UB, Morgenthaler JJ. Fibronectin-cleaving activity in bronchial secretions of patients with cystic fibrosis. J Infect Dis 1988;158:89-100.
17. 1-antitrypsin, emphysema and smoking. NZ Med J 1984;97:327.
18. Belaaouaj A, McCarthy R, Baumann M, et al. Mice lacking neutrophil elastase reveal impaired host defence against gram negative bacterial sepsis. Nature Med 1998;4:615-8.
19. Döring G. The role of neutrophil elastase in chronic inflammation. Am J Respir Crit Care Med 1994;150:s114-7.
20. Döring G, Goldstein W, Bozenhart K, et al. Elastase from polymorphonuclear leucocytes: a regulatory enzyme in immune complex disease. Clin Exp Immunol 1986;64:597-605.
21. Trevani AS, Andonegui G, Giordano M, et al. Neutrophil apoptosis induced by proteolytic enzymes. Lab Invest 1996; 74:711-21.