Alpha 1-Antitrypsin deficiency : biology, pathogenesis, clinical manifestations, therapy

Bibliographic Information

Alpha 1-Antitrypsin deficiency : biology, pathogenesis, clinical manifestations, therapy

edited by Ronald G. Crystal

(Lung biology in health and disease, v. 88)

Marcel Dekker, c1996

Available at  / 15 libraries

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Includes bibliographical references and indexes

Description and Table of Contents

Description

Providing 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 Contents

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, Frederic 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

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