Alpha 1-antitrypsin
- OMIM: 107400
- UniProt: P01009
- MGI: 891 971
- CAS Number: 9041-92-3
α1 -antitrypsin (AAT ) or α1 - protease inhibitor ( α1PI ) is an acute phase protein, protects against the body tissue involved in the inflammatory process enzymes.
Description
AAT acts primarily by inhibiting PMN - elastase, trypsin and other serine proteases, but also. If this inhibition by a lack or limited in the function of AAT, elastase example, picks up on the connective tissue of the lungs, which can lead to serious complications.
An increase in the α1 -antitrypsin value occurs in at
- Acute exacerbation of chronic inflammatory processes,
- Tumors (especially lung cancer, see tumor markers ),
- Pregnancy or
- Estrogen treatment
A reduction of the α1 -antitrypsin value is given in
- Hereditary α1 - antitrypsin deficiency,
- Prolongatus jaundice and hepatitis of the newborn,
- Childlike chronic hepatitis or liver cirrhosis or
- Emphysema
In the AAT deficiency occurs primarily by proteases in the loss of connective tissue and function in the lung. This results in a malfunction with similarity to chronic obstructive pulmonary disease (COPD) and emphysema in particular. This course can lead to death by respiratory failure. The severity of the illness may be brought technically computed tomography of the lung, lung function ( FEV1 particular ) and collection of blood gases.
The normal concentration of α1 -antitrypsin in the blood plasma is from 83 to 199 mg / dl. In the course of inflammatory processes, the value increases. In comparison with the C- reactive protein, the activity measurement of AAT is a better marker to monitor the course of inflammation. If you suspect a deficiency of AAT simultaneous determination of C-reactive protein is useful because inflammation can hide the defect.
Therapy
First, a therapy is as in COPD into consideration. Furthermore, intravenous AAT substitution can occur if the disease is not too advanced Newer therapeutic approaches can also be an inhalation of AAT make sense.