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Biochemistry of Pulmonary Emphysema pp 159–167Cite as

Genetic Control of Human Alpha-1-Antitrypsin and Hepatic Gene Therapy

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Part of the book series: Current Topics in Rehabilitation ((CURRENT REHAB))

Abstract

Alpha-1-antitrypsin (AAT) is a 50 kDa serine protease inhibitor which is synthesized most abundantly by hepatocytes.1,2 It accounts for approximately 90% of the total protease inhibitory capacity in normal human serum.3,4 In common with other members of the serine protease inhibitor superfamily,5 the active inhibitory site of AAT is centred6 around Ser358. Although it exhibits the capacity to inhibit a variety of proteases, including trypsin, chymotrypsin, thrombin, kallikrein, and plasmin7, its apparent major physiological role is to diffuse into the alveoli structure of the lung, and protect elastin fibres from excessive hydrolysis by neutrophil elastase.8,9 Neutrophil elastase is inhibited by forming a pseudo-irreversible equimolar complex of the protease with inhibitor.10

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Author information

Authors and Affiliations

  1. Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas, USA

    S. L. C. Woo

  2. Department of Cell Biology, Baylor College of Medicine, Houston, Texas, USA

    S. L. C. Woo, R. N. Sifers & K. Ponder

  3. Department of Pathology, Baylor College of Medicine, Houston, Texas, USA

    R. N. Sifers

Authors
  1. S. L. C. Woo
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  2. R. N. Sifers
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  3. K. Ponder
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Editor information

Editors and Affiliations

  1. Institute of Phthisiology and Respiratory Diseases, IRCCS San Matteo, University of Pavia, Italy

    C. Grassi  & M. Luisetti  & 

  2. Department of Biochemistry, University of Georgia, Athens, GA, USA

    J. Travis

  3. Postgraduate School of Respiratory Diseases, University of Pavia, Italy

    L. Casali

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© 1992 Springer-Verlag London

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Woo, S.L.C., Sifers, R.N., Ponder, K. (1992). Genetic Control of Human Alpha-1-Antitrypsin and Hepatic Gene Therapy. In: Grassi, C., Travis, J., Casali, L., Luisetti, M. (eds) Biochemistry of Pulmonary Emphysema. Current Topics in Rehabilitation. Springer, London. https://doi.org/10.1007/978-1-4471-3771-9_12

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  • DOI: https://doi.org/10.1007/978-1-4471-3771-9_12

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-3773-3

  • Online ISBN: 978-1-4471-3771-9

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