The most common indications for LT– accounting for ~85% of the proceduresperformed worldwide – are advanced chronicobstructive pulmonary disease (COPD),idiopathic pulmonary fibrosis (IPF), cysticfibrosis (CF), emphysema due to alpha1-antitrypsin deficiency and pulmonaryarterial hypertension (PAH). The gene discussed is SERPINA1; the disease is cystic fibrosis.