Alpha-1 Antitrypsin Deficiency

Chapter 1 Pathophysiology of Alpha-1 Antitrypsin Deficiency Liver Disease

Chapter 2 Pathophysiology of Alpha-1 Antitrypsin Lung Disease

Chapter 3 Measuring and Interpreting Serum AAT Concentration

Chapter 4 AAT Phenotype Identification by Isoelectric Focusing

Chapter 5 Laboratory Diagnosis by Genotyping

Chapter 6 Genotyping Protocol for the Alpha-1 Antitrypsin (PiZ) Mouse Model

Chapter 7 Elastase-Induced Lung Emphysema Models in Mice

Chapter 8 Assessing Structure–Function Relations in Mice Using the Forced Oscillation Technique and Quantitative Histology

Chapter 9 Practical Methods for Assessing Emphysema Severity Based on Estimation of Linear Mean Intercept (Lm) in the Context of Animal Models of Alpha-1 Antitrypsin Deficiency

Chapter 10 Design, Cloning, and In Vitro Screening of Artificial miRNAs to Silence Alpha-1 Antitrypsin

Chapter 11 Methods to Identify and Characterize siRNAs Targeting Serpin A1 In Vitro and In Vivo Using RNA Interference

Chapter 12 Knockdown of Z Mutant Alpha-1 Antitrypsin In Vivo Using Modified DNA Antisense Oligonucleotides

Chapter 13 Immunohistochemistry Staining for Human Alpha-1 Antitrypsin

Chapter 14 Periodic Acid-Schiff Staining with Diastase

Chapter 15 Protocol for Directed Differentiation of Human Induced Pluripotent Stem Cells (iPSCs) to a Hepatic Lineage

Chapter 16 Isolation of Kupffer Cells and Hepatocytes from a Single Mouse Liver

Chapter 17 Alpha-1 Antitrypsin Transcytosis and Secretion

Chapter 18 Measuring the Effect of Histone Deacetylase Inhibitors (HDACi) on the Secretion and Activity of Alpha-1 Antitrypsin

Chapter 19 Expression and Purification of Active Recombinant Human Alpha-1 Antitrypsin (AAT) from Escherichia coli

Chapter 20 Quantification of Total Human Alpha-1 Antitrypsin by Sandwich ELISA

Chapter 21 Quantification of Murine AAT by Direct ELISA

Chapter 22 Quantification of Z-AAT by a Z-Specific “Sandwich” ELISA

Chapter 23 Semiquantitation of Monomer and Polymer Alpha-1 Antitrypsin by Centrifugal Separation and Assay by Western Blot of Soluble and Insoluble Components

Chapter 24 Electrophoresis- and FRET-Based Measures of Serpin Polymerization

Chapter 25 Therapeutics: Alpha-1 Antitrypsin Augmentation Therapy

Chapter 26 Therapeutic Options in Alpha-1 Antitrypsin Deficiency: Liver Transplantation

Chapter 27 Therapeutics: Gene Therapy for Alpha-1 Antitrypsin Deficiency