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Alpha-1 Antitrypsin Deficiency

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Cover of 'Alpha-1 Antitrypsin Deficiency'

Table of Contents

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    Book Overview
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    Chapter 1 Pathophysiology of Alpha-1 Antitrypsin Deficiency Liver Disease
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    Chapter 2 Pathophysiology of Alpha-1 Antitrypsin Lung Disease
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    Chapter 3 Measuring and Interpreting Serum AAT Concentration
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    Chapter 4 AAT Phenotype Identification by Isoelectric Focusing
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    Chapter 5 Laboratory Diagnosis by Genotyping
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    Chapter 6 Genotyping Protocol for the Alpha-1 Antitrypsin (PiZ) Mouse Model
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    Chapter 7 Elastase-Induced Lung Emphysema Models in Mice
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    Chapter 8 Assessing Structure–Function Relations in Mice Using the Forced Oscillation Technique and Quantitative Histology
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    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
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    Chapter 10 Design, Cloning, and In Vitro Screening of Artificial miRNAs to Silence Alpha-1 Antitrypsin
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    Chapter 11 Methods to Identify and Characterize siRNAs Targeting Serpin A1 In Vitro and In Vivo Using RNA Interference
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    Chapter 12 Knockdown of Z Mutant Alpha-1 Antitrypsin In Vivo Using Modified DNA Antisense Oligonucleotides
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    Chapter 13 Immunohistochemistry Staining for Human Alpha-1 Antitrypsin
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    Chapter 14 Periodic Acid-Schiff Staining with Diastase
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    Chapter 15 Protocol for Directed Differentiation of Human Induced Pluripotent Stem Cells (iPSCs) to a Hepatic Lineage
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    Chapter 16 Isolation of Kupffer Cells and Hepatocytes from a Single Mouse Liver
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    Chapter 17 Alpha-1 Antitrypsin Transcytosis and Secretion
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    Chapter 18 Measuring the Effect of Histone Deacetylase Inhibitors (HDACi) on the Secretion and Activity of Alpha-1 Antitrypsin
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    Chapter 19 Expression and Purification of Active Recombinant Human Alpha-1 Antitrypsin (AAT) from Escherichia coli
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    Chapter 20 Quantification of Total Human Alpha-1 Antitrypsin by Sandwich ELISA
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    Chapter 21 Quantification of Murine AAT by Direct ELISA
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    Chapter 22 Quantification of Z-AAT by a Z-Specific “Sandwich” ELISA
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    Chapter 23 Semiquantitation of Monomer and Polymer Alpha-1 Antitrypsin by Centrifugal Separation and Assay by Western Blot of Soluble and Insoluble Components
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    Chapter 24 Electrophoresis- and FRET-Based Measures of Serpin Polymerization
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    Chapter 25 Therapeutics: Alpha-1 Antitrypsin Augmentation Therapy
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    Chapter 26 Therapeutic Options in Alpha-1 Antitrypsin Deficiency: Liver Transplantation
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    Chapter 27 Therapeutics: Gene Therapy for Alpha-1 Antitrypsin Deficiency
Attention for Chapter 7: Elastase-Induced Lung Emphysema Models in Mice
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Chapter title
Elastase-Induced Lung Emphysema Models in Mice
Chapter number 7
Book title
Alpha-1 Antitrypsin Deficiency
Published in
Methods in molecular biology, January 2017
DOI 10.1007/978-1-4939-7163-3_7
Pubmed ID
Book ISBNs
978-1-4939-7161-9, 978-1-4939-7163-3
Authors

Béla Suki, Erzsébet Bartolák-Suki, Patricia R. M. Rocco, Suki, Béla, Bartolák-Suki, Erzsébet, Rocco, Patricia R. M.

Abstract

Pulmonary emphysema is one of the distinct pathological forms of chronic obstructive pulmonary disease (COPD) that is accompanied by gradual elimination of alveolar tissue, causing reductions in lung recoil and leading to difficulty in breathing. As there is no cure for emphysema, animal models are often used to better understand the pathogenesis and progression of the disease. One widely used animal model of emphysema is the elastase treatment. In this chapter, we describe two methods of elastase-induced emphysema in mice. The first is a single-dose treatment, whereby elastase is introduced oropharengeally into the lung and the structure and/or function of the lungs are studied between 2 days and 4 weeks following the treatment. The second method consists of exposing mice repeatedly (four times) to elastase intratracheally and observing the effects of the treatment 1-4 weeks following the last administration of the enzyme. Both protocols are described in detail, and examples of lung structure and function of the emphysematous mouse lung are provided.

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 41 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 41 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 8 20%
Student > Bachelor 6 15%
Student > Master 6 15%
Researcher 4 10%
Student > Doctoral Student 1 2%
Other 4 10%
Unknown 12 29%
Readers by discipline Count As %
Medicine and Dentistry 5 12%
Biochemistry, Genetics and Molecular Biology 5 12%
Pharmacology, Toxicology and Pharmaceutical Science 3 7%
Chemistry 3 7%
Immunology and Microbiology 3 7%
Other 8 20%
Unknown 14 34%