1st Edition

Alpha 1 - Antitrypsin Deficiency Biology-Pathogenesis-Clinical Manifestations-Therapy

By Crystal Copyright 1996

    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.


    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


    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


    Ronald G. Crystal