New Insights into the Biology of 1-Antitrypsin and its Role in Chronic Obstructive Pulmonary Disease

Author(s): Sabina M. Janciauskiene, Tim Stevens, Ignacio Blanco

Journal Name: Current Respiratory Medicine Reviews

Volume 3 , Issue 2 , 2007

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α1-antitrypsin, an acute phase protein, is the prototypic member of the serpin super family and a major inhibitor of serine proteases. As an acute phase protein, α1-antitrypsin is thought to play an important role in limiting host tissue injury at sites of inflammation. The clinical importance of α1-antitrypsin is highlighted in individuals with inherited α1- antitrypsin deficiency who exhibit an increased susceptibility to develop chronic inflammatory conditions including chronic obstructive pulmonary disease, systemic vasculitis and necrotizing panniculitis. There is now an increasing evidence that α1-antitrypsin may also exhibit biological activity independent of its protease inhibitor function. Thus, conformationally modified and degraded forms of α1-antitrypsin, which lack antiprotease activity, demonstrate specific biological effects in vitro and in vivo and highlight the potentially broader modulatory role of α1-antitrypsin in inflammatory diseases. In this review we discuss the biological properties of α1-antitrypsin and its role in chronic obstructive pulmonary diseases. A more comprehensive understanding of the biology of native α1-antitrypsin and its modified forms may have a significant impact on our understanding and ultimately treatment of disease pathologies arising from both natural point mutations and from post-synthetically modified byproducts of α1-antitrypsin.

Keywords: Serpins, α1-antitrypsin, deficiency, chronic inflammatory diseases, COPD

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Article Details

Year: 2007
Page: [147 - 158]
Pages: 12
DOI: 10.2174/157339807780599153
Price: $65

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