Alpha-1-antitrypsin Deficiency

Alpha-1-antitrypsin Deficiency

Biology, Diagnosis, Clinical Significance, and Emerging Therapies

1st Edition - June 6, 2017

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  • Authors: Noor Kalsheker, Robert Stockley
  • eBook ISBN: 9780128039472
  • Paperback ISBN: 9780128039427

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Alpha-1-antitrypsin Deficiency: Biology, Diagnosis, Clinical Significance, and Emerging Therapies is the authoritative reference on AATD, providing standards for diagnosis, monitoring, treatment and appropriate avenues of research. The book covers the disease from basic biology and epidemiology, to clinical impact, and includes the understanding of the natural history of the disease and the significant advances that have been made in the last 20 years, including the three-dimensional structure of the molecule, its broad biological activity and improved therapeutic options, including replacement therapy and gene therapy. The editors have recruited international experts in the field to contribute evidence-based chapters and insights on future developments in the understanding of this disease.

Key Features

  • Provides documentation of the variations in clinical presentation and pathology in a single reference
  • Presents new insights by pulling together the advances in the understanding of the structure and function of alpha1-antitrypsin deficiency with the genetic variants that cause the disease
  • Allows for easy reference for the diagnosis of AATD to lead to better therapeutics


Scientists and clinicians involved in respiratory research, respiratory physicians, hepatologists, dermatologists, renal physicians, radiologists, general physicians

Table of Contents

  • 1. Historical perspective
    2. The Swedish AAT screening study: What we have learnt-from birth through to young middle age
    3. Gene regulation
    4. AAT variants
    5. AAT structure and function
    6. Novel biological functions of A1AT
    7. AATD and lung disease
    8. Alpha-1-Antitrypsin Deficiency Liver Disease
    9. AATD epidemiological studies and other AATD associated diseases
    11. Animal models of alpha-1 antitrypsin deficiency
    12. Establishing European Registries for AATD and clinical trials for lung disease
    13. Establishing the USA Registry: logistics, impact and early clinical trials
    14. Gene therapy
    15. The future of AAT research

Product details

  • No. of pages: 262
  • Language: English
  • Copyright: © Academic Press 2017
  • Published: June 6, 2017
  • Imprint: Academic Press
  • eBook ISBN: 9780128039472
  • Paperback ISBN: 9780128039427

About the Authors

Noor Kalsheker

Professor Kalsheker is the Head of Division and Co-director of the Institute of Genetics and teaches in the School of Life Sciences at the University of Nottingham. His work is focused on the study of humoral control of genes of the serine proteinase inhibitor family which regulate key biological pathways and how functional genetic variants contribute to complex diseases such as chronic lung disease.

Affiliations and Expertise

Professor, The University of Nottingham, School of Life Sciences, Nottingham, UK

Robert Stockley

Robert Stockley
Professor Stockley has a longstanding interest in chronic obstructive pulmonary disease (COPD) with special reference to airway inflammation, proteinases and anti-proteinases, and especially the role of the neutrophils, bacteria and exacerbations. His research focuses on investigating the factors governing neutrophil differentiation, activation and recruitment as processes that determine the degree of tissue damage resulting in the establishment and progression of chronic lung disease.

Affiliations and Expertise

Professor of Medicine, Birmingham University; Director of Research and Development, University Hospital Birmingham NHS Foundation Trust; Medical Director, Lung Resource Centre, Queen Elizabeth Hospital Birmingham Birmingham, UK

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