Introduction --Alpha 1-Antitrypsin Deficiency: An Introduction, Andrea Mastrangeli and Ronald G. Crystal -- Biology and Pathobiology --Alpha 1-Antitrypsin Gene and Promoter, John G. Hay, Motoyoshi Suzuki, and Ronald G. Crystal--Alpha 1-Antitrypsin Gene Evolution, Toshihiro Nukiwa, Fumitaka Ogushi, and Ronald G. Crystal--Alpha 1-Antitrypsin Genotypes and Phenotypes, Mark Brantly--Structure and Expression of the Human Neutrophil Elastase Gene, Kunihiko Yoshimura--The Three-Dimensional Structure of a1AT, Richard A. Engh and Wolfman Bode--The Three-Dimensional Structure of Human Neutrophil Elastase, Wolfram Bode--Kinetics of Neutrophil Elastase Inhibition In Vivo, Joseph G. Bieth--Alpha 1-Antitrypsin Gene Expression in Neutrophils and Other Cells, Roland M. du Bois--Alpha 1-Antitrypsin Gene Expression in Hepatocytes, Alfredo Nicosia and Paola Monaci--Alpha 1-Antitrypsin Gene Expression in Mononuclear Phagocytes, Gregg Joslin and David H. Perlmutter--Transgenic Animals with the a1AT Promoter, Wilfried Dalemans, Frdric Perraud, Dalila Ali-Hadji, Sophie Jallat, and Andrea Pavirani--Chemistry and Biology of Secretory Leukoprotease Inhibitor, David F. Carmichael and Kjell Ohlsson -- Clinical Manifestations --Laboratory Diagnosis of a1AT Deficiency, Mark Brantly--Clinical Manifestations of a1AT Deficiency, Noel G. McElvaney and Ronald G. Crystal--Lung Function and a1AT Deficiency, Alan F. Barker, Ray G. D'silva, and A. Sonia Buist--Reactive Airways Disease and a1AT Deficiency, Robert J. Fallat--Natural History of a1AT Deficiency, Janet Wittes and Margaret C. Wu--The Prevalence of a1AT Deficiency Outside the United States and Europe, Toshihiro Nukiwa, Kuniaki Seyama, and Shiro Kira--The National Institutes of Health National Heart, Lung and Blood Institute Registry for Patients with Severe Deficiency of Alpha 1-Antitrypsin, James K. Stoller -- Treatment --Therapy of a1AT Deficiency, Noel G. McElvaney and Robert G. Crystal--Augmentation of Liver Production, Mark D. Wewers--Low-Molecular-Weight Inhibitors of Neutrophil Elastase, James C. Powers, R. Richard Plaskon, and Chih-Min Kam--a1AT Deficiency and Liver Transplantation, David H. Van Thiel and Thomas E. Starzl--Lung Transplantation for a1AT Deficiency Emphysema, Elbert P. Trulock and Joel D. Cooper.