Alpha-1-antitrypsin deficiency
A relationship between the development of emphysema and low serum levels of alpha-1-antitrypsin (AAT) was first noted in 1963 by Laurell and Eriksson [1]. A few years later, Sharp et al. [2] reported liver damage in AAT deficiency (AATD) AAT, also referred to as αl-protease inhibitor or protein inhi...
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| Main Author: | |
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| Format: | Chapter/Article |
| Language: | English |
| Published: |
1995
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| In: |
Inborn metabolic diseases
Year: 1995, Pages: 361-363 |
| DOI: | 10.1007/978-3-662-03147-6_33 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1007/978-3-662-03147-6_33 Verlag: https://link.springer.com/chapter/10.1007/978-3-662-03147-6_33 
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| Author Notes: | D.J.F. Feist |
MARC
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| 520 | |a A relationship between the development of emphysema and low serum levels of alpha-1-antitrypsin (AAT) was first noted in 1963 by Laurell and Eriksson [1]. A few years later, Sharp et al. [2] reported liver damage in AAT deficiency (AATD) AAT, also referred to as αl-protease inhibitor or protein inhibitor (PI), is a 52-kDa glycoprotein synthesized in the liver and secreted into plasma, where it accounts for 90% of serum α1-globulin. Although inhibitory of trypsin (hence its name), the major function of AAT is to inhibit neutrophil elastase at inflammation sites, e.g., within the alveoles. Whereas normal AAT (termed M) readily diffuses throughout interstitial fluids, the defective molecules formed in AATD aggregate inside the hepatocytes, resulting in poor secretion into blood and the fluids of the respiratory tract. Several defective variants of AAT have been identified, of which the most frequently encountered is Z. | ||
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