Alpha 1 - Antitrypsin Deficiency: Biology-Pathogenesis-Clinical Manifestations-Therapy, 1st Edition (Hardback) book cover

Alpha 1 - Antitrypsin Deficiency

Biology-Pathogenesis-Clinical Manifestations-Therapy, 1st Edition

By Crystal

CRC Press

472 pages

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Hardback: 9780824788483
pub: 1995-10-26
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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


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

About the Series

Lung Biology in Health and Disease

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Subject Categories

BISAC Subject Codes/Headings:
MEDICAL / General
MEDICAL / Biochemistry
MEDICAL / Internal Medicine
MEDICAL / Pulmonary & Thoracic Medicine