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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 22: Quantification of Z-AAT by a Z-Specific “Sandwich” ELISA
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Chapter title
Quantification of Z-AAT by a Z-Specific “Sandwich” ELISA
Chapter number 22
Book title
Alpha-1 Antitrypsin Deficiency
Published in
Methods in molecular biology, January 2017
DOI 10.1007/978-1-4939-7163-3_22
Pubmed ID
Book ISBNs
978-1-4939-7161-9, 978-1-4939-7163-3
Authors

Florie Borel, Qiushi Tang, Christian Mueller

Abstract

This protocol describes an enzyme-linked immunosorbent assay (ELISA) to specifically detect Z-alpha-1 antitrypsin (AAT), the most common protein variant associated with alpha-1 antitrypsin deficiency. This "sandwich" ELISA relies on an anti-Z-AAT specific capture antibody and a HRP-conjugated anti-AAT detection antibody. This method would be of interest to identify and quantify Z-AAT in a variety of samples such as cell culture medium, cell or tissue lysate, animal or patient serum. Because this method is specific and sensitive, it would be particularly valuable for detection of Z-AAT in the presence of background M-AAT, for instance when quantifying silencing of Z-AAT in patients undergoing M-AAT augmentation therapy.

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 10 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 10 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 3 30%
Unspecified 1 10%
Professor > Associate Professor 1 10%
Student > Postgraduate 1 10%
Unknown 4 40%
Readers by discipline Count As %
Unspecified 1 10%
Pharmacology, Toxicology and Pharmaceutical Science 1 10%
Biochemistry, Genetics and Molecular Biology 1 10%
Agricultural and Biological Sciences 1 10%
Unknown 6 60%