<unk> <unk><unk>  (A1AT) is synthesized and secreted mainly by hepatocytes.<unk><unk><unk>  enzyme is a member of the serine protease inhibitor (serpin) family of proteins.<unk> <unk><unk>  (A1AT) protects the lungs from proteases like the neutrophil elastase enzyme. A genetic mutation in the SERPINA1 gene results in decreased levels of alveolar<unk><unk><unk> . Proteases accumulate in the alveoli causing a destruction of alveolar walls and resultant emphysema. Acculmulation of excess<unk><unk><unk>  in hepatocytes results in chronic liver disease. SERPINA1 gene mutation alters the structure of the<unk><unk><unk>  molecule and prevents its release from hepatocytes. By far, the most common severe deficient variant is the Z allele, which is produced by lysine substitution for glutamate at 342 position in the<unk> <unk><unk>  molecule.  The Z allele accounts for 95% of the clinically recognized cases of severe<unk> -1 AT<unk> . On cut section of the lung, emphysematous process is evidenced by dilated air spaces and loss of lung parenchyma. Superimposed infections can result in scarring. Panacinar emphysema is commonly associated with AATD with loss of all portions of the acinus from the respiratory bronchiole to the alveoli. In<unk><unk><unk><unk>  (AATD), the emphysematous areas are uniformly distributed throughout the lobule found more commonly in the basilar portions of the lung.
Alpha 1-antitrypsin deficiency pathophysiology
Farmer's lung pathophysiology
Emphysema pathophysiology
Idiopathic pulmonary fibrosis risk factors
Acute bronchitis pathophysiology
Pneumoconiosis pathophysiology
Bronchiolitis pathophysiology
Tracheitis pathophysiology