Description
Book SynopsisProviding a broad overview of basic and clinical aspects of alpha 1-antitrypsin (a 1AT) deficiency, this up-to-date reference discusses the complex pathobiological processes underlying the pathogenesis of a1AT deficiency, describes the a1AT gene and its promoter, and details specific therapies to prevent the major clinical manifestations of the disorder.
Table of ContentsIntroduction
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, Frédéric 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
