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When a pathogen is detected in the lungs, neutrophils release elastase, an enzyme that degrades bacterial cell walls and disrupts biofilms. However, elastase has to be neutralized quickly because it also starts to break down the elastin and collagen in the connective tissue of the lungs. The liver secretes a serine protease called alpha-1 antitrypsin that degrades elastase and protects the lungs. In alpha-1 antitrypsin deficiency, a mutation in the SERPIN1A gene prevents the liver from secreting alpha-1 antitrypsin, leading to emphysema and chronic obstructive pulmonary disease. Accumulation of misfolded antitrypsin proteins in the liver also causes ER stress and can result in cirrhosis or liver cancer. In severe cases, transplantation of the lungs or liver may be needed. 00:00 Introduction 00:14 Alveoli of the lungs 01:16 Glycans 03:02 ABO blood groups 03:53 Carbohydrate digestion 04:11 Cell surface glycoproteins 05:34 Glycogen 06:24 N-linked glycoproteins 08:32 Quality control 09:45 Golgi apparatus 11:01 Pathogenesis of AATD 11:50 Treatment 12:46 Summary Disclaimer: This video has been made available without warranty for informational and educational purposes only and is not intended to substitute for the advice of a medical professional.