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.
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.
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 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
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 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
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