Strategies for aerosol therapy ofα 1-antitrypsin deficiency by the aerosol route (original) (raw)
Gadek JE, Crystal RG (1982) Alpha 1-antitrypsin deficiency. In: Stanbury JB, Wyngaarden JB, Frederickson DS, Goldstein JL, Brown MS (eds): The metabolic basis of inherted disease. McGraw-Hill, New York, pp 1450–1467 Google Scholar
Tobin MJ, Cook PJ, Hutchison CS (1983) Alpha 1-antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest 77:14–27 ArticlePubMedCAS Google Scholar
More JO (1978) Alpha 1-antitrypsin deficiency. N Engl J Med 299:1045–1048, 1099–1105 Article Google Scholar
Hubbard RC, Crystal RG (1988) Alpha-1-antitypsin augmentation therapy for alpha-1-antitrypsin deficiency. Am J Med 84:52–62 PubMedCAS Google Scholar
Brantly M, Nukiwa T, Crystal RG (1988) Molecular basis of alpha-1-antitrypsin deficiency. Am J Med 84:13–31 PubMed Google Scholar
Gadek JE, Fells GA, Zimmerman RL, Rennard SI, Crystal RG (1981) Antielastases of the human alveolar structures: implications for the protease-antiprotease theory of emphysema. J Clin Invest 68:889–898 ArticlePubMedCAS Google Scholar
Janoff A (1985) Elastases and emphysema: current assessment of the protease-antiprotease hypothesis. Am Rev Respir Dis 132:417–433 PubMedCAS Google Scholar
Stockley RA (1983) Proteolytic enzymes, their inhibitors and lung diseases. Clin Sci 64:119–126 PubMedCAS Google Scholar
Wewers MD, Casolaro MA, Sellers SE, Swayze SC, McPhaul K, Wittes JT, Crystal RG. (1987) Replacement therapy for alpha-1-antitrypsin deficiency associated with emphysema. N Engl J Med 316:1055–1062 ArticlePubMedCAS Google Scholar
Hubbard RC, Sellers S, Czerski D, Stephens L, Crystal RG (1988) Biochemical efficacy and safety of monthly augmentation therapy of alpha-1-antitrypsin deficiency. JAMA 260:1259–1264 ArticlePubMedCAS Google Scholar
Hubbard RC, Casolaro MA, Mitchell M, Sellers SE, Matthay MA, Crystal RG (1989) Fate of aerosolized recombinant DNA produced_α_1-antitrypsin: use of the epitheial surface of the lower respiratory tract to administer proteins of therapeutic importance. Proc Natl Acad Sci 86:680–684 ArticlePubMedCAS Google Scholar
Hubbard RC, Brantly ML, Sellers SE, Mitchell ME, Crystal RG (1989) Delivery of proteins for therapeutic purposes by aerosolization: direct augmentation of anti-neutrophil elastase defenses of the lower respiratory tract in_α_1-antitrypsin deficiency with an aerosol of_α_1-antitrypsin. Ann Intern Med 111:206–212 PubMedCAS Google Scholar
Hubbard RC, McElvaney NG, Sellers SE, Healy JT, Czerski DB, Crystal RG (1989) Recombinant DNA-produced_α_1-antitrypsin administered by aerosol augment lower respiratory tract anti-neutrophil elastase defenses in individuals with_α_1-antitrypsin deficiency. J Clin Invest 84:1349–1354 ArticlePubMedCAS Google Scholar
Weibel ER (1980) Design and structure of human lung. In: Fishman AP (ed) Pulmonary diseases and disorders. McGraw-Hill, New York, pp 224–272 Google Scholar
Bieth JG (1986) Elastases: catalytic and biological properties. In: Mechan R (ed) Regulation of matrix accumulation. Academic Press, New York, pp 217–320 Google Scholar
Gorin AB, Stewart PA (1979) Differential permeability of endothelial and epithelial barriers to albumin flux. J Appl Physiol 47:1315–1324 PubMedCAS Google Scholar
Matthay MA, Berthiaume Y, Staub NC (1985) Long-term clearance of liquid and protein from the lungs of unanesthetized sheep. J Appl Physiol 59:928–934 PubMedCAS Google Scholar
Crystal RG, Brantly ML, Hubbard RC, Curiel DT, States DJ, Holmes MD: (1989) The_α_1-antitrypsin gene and its mutations: clinical consequences and strategies for therapy. Chest 95:196–208 ArticlePubMedCAS Google Scholar
Coan MH, Brockway WJ, Equizabal H, Krieg T, Fournel M (1985) Preparation and properties of alpha 1-proteinase inhibitor concentrate from human plasma. Vox Sang 48:333–342 ArticlePubMedCAS Google Scholar
Beatty K, Bieth J, Travis J (1980) Kinetics of association of serine proteinases with native and oxidized alpha-1-proteinase inhibitor and_α_-1-antichymotrypsin. J Biol Chem 255:3931–3934 PubMedCAS Google Scholar
Ogushi F, Fells GA, Hubbard RC, Straus SD, Crystal RG (1987) Z-type_α_1-antitrypsin is less competent than M 1-type_α_1-antitrypsin as an inhibitor of neutrophil elastase. J Clin Invest 80:1366–1374 ArticlePubMedCAS Google Scholar
Casolaro MA, Fells G, Wewers M, Pierce JE, Ogushi F, Hubbard R, Sellers S, Forstrom J, Lyons D, Kawasaki G, Crystal RG (1987) Augmentation of lung antineutrophil elastase capacity with recombinant human_α_1-antitrypsin. J Appl Physiol 63:2015–2023 PubMedCAS Google Scholar
Smith RM, Spragg RG (1988) Production and administration to dogs of aerosols of alpha-1-proteinase inhibitor. Am J Med 84:48–51 PubMed Google Scholar
Courtney M, Jallat A, Tessier L-H, Benavente A, Crystal RG, LeCocq JP (1985) Synthesis in E. coli of alpha 1-antitrypsin variants with potential in the therapy of emphysema and thrombosis. Nature 313:149–151 ArticlePubMedCAS Google Scholar
Hubbard RC, Ogushi F, Fells GA, Cantin AM, Courtney M, Crystal RG (1987) Oxidants spontaneously released by alveolar macrophages of cigarette smokers can inactivate the active site of_α_1-antitrypsin, rendering it ineffective as an inhibitor of neutrophil elastase. J Clin Invest 80:1289–1295 ArticlePubMedCAS Google Scholar
Agnew JE (1984) Physical properties and mechanisms of deposition of aerosols. In: Clark SW, Pavia D (eds) Aerosols and the lung: clinical and experimental aspects. Butterworths, London, pp 49–70 Google Scholar