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Alpha-1 Antitrypsin Deficiency

Methods and Protocols

  • Book
  • © 2017

Overview

Editors:
  1. Florie Borel

    1. Horae Gene Therapy Center, University of Massachusetts Medical School, Worcester, USA

    View editor publications

    You can also search for this editor in PubMed   Google Scholar

  2. Christian Mueller

    1. Horae Gene Therapy Center, University of Massachusetts Medical School, Worcester, USA

    View editor publications

    You can also search for this editor in PubMed   Google Scholar

  • Includes cutting-edge methods and protocols.
  • Provides step-by-step detail essential for reproducible results.
  • Contains key notes and implementation advice from the experts
  • Includes supplementary material: sn.pub/extras
  • Includes supplementary material: sn.pub/extras

Part of the book series: Methods in Molecular Biology (MIMB, volume 1639)

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Table of contents (27 protocols)

  1. Front Matter

    Pages i-xiii
    Download chapter PDF

  2. Pathophysiology of Alpha-1 Antitrypsin Deficiency Liver Disease

    • Jeffrey H. Teckman, Keith S. Blomenkamp
    Pages 1-8

  3. Pathophysiology of Alpha-1 Antitrypsin Lung Disease

    • Michael Kalfopoulos, Kaitlyn Wetmore, Mai K. ElMallah
    Pages 9-19

  4. Measuring and Interpreting Serum AAT Concentration

    • Leslie J. Donato, Melissa R. Snyder, Dina N. Greene
    Pages 21-32

  5. AAT Phenotype Identification by Isoelectric Focusing

    • Dina N. Greene, M. C. Elliott-Jelf, David G. Grenache
    Pages 33-44

  6. Laboratory Diagnosis by Genotyping

    • Irene Belmonte, Luciana Montoto, Francisco Rodríguez-Frías
    Pages 45-60

  7. Genotyping Protocol for the Alpha-1 Antitrypsin (PiZ) Mouse Model

    • Alisha M. Gruntman
    Pages 61-65

  8. Elastase-Induced Lung Emphysema Models in Mice

    • Béla Suki, Erzsébet Bartolák-Suki, Patricia R. M. Rocco
    Pages 67-75

  9. Assessing Structure–Function Relations in Mice Using the Forced Oscillation Technique and Quantitative Histology

    • Harikrishnan Parameswaran, Béla Suki
    Pages 77-91

  10. 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

    • Airiel M. Davis, Kristen E. Thane, Andrew M. Hoffman
    Pages 93-106

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

    • Florie Borel, Christian Mueller
    Pages 107-114

  12. Methods to Identify and Characterize siRNAs Targeting Serpin A1 In Vitro and In Vivo Using RNA Interference

    • Stuart Milstein, Kun Qian, Linying Zhang, Alfica Sehgal
    Pages 115-126

  13. Knockdown of Z Mutant Alpha-1 Antitrypsin In Vivo Using Modified DNA Antisense Oligonucleotides

    • Mariam Aghajan, Shuling Guo, Brett P. Monia
    Pages 127-138

  14. Immunohistochemistry Staining for Human Alpha-1 Antitrypsin

    • Dongtao A. Fu, Martha Campbell-Thompson
    Pages 139-143

  15. Periodic Acid-Schiff Staining with Diastase

    • Dongtao A. Fu, Martha Campbell-Thompson
    Pages 145-149

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

    • Joseph E. Kaserman, Andrew A. Wilson
    Pages 151-160

  17. Isolation of Kupffer Cells and Hepatocytes from a Single Mouse Liver

    • Marcela Aparicio-Vergara, Michaela Tencerova, Cecilia Morgantini, Emelie Barreby, Myriam Aouadi
    Pages 161-171

  18. Alpha-1 Antitrypsin Transcytosis and Secretion

    • Angelia D. Lockett
    Pages 173-184

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

    • Chao Wang, Marion Bouchecareilh, William E. Balch
    Pages 185-193

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

    • Beena Krishnan, Lizbeth Hedstrom, Daniel N. Hebert, Lila M. Gierasch, Anne Gershenson
    Pages 195-209
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Keywords

About this book

This volume provides protocols that expand on the latest alpha-1-antitrypsin (AAT) research. The chapters in this book are divided in to three sections: Part I is dedicated to patient-oriented research; part II discusses animal models; and Part III focuses on in vitro studies. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls.

Cutting-edge and authoritative, Alpha-1 Antitrypsin Deficiency: Methods and Protocols is a valuable resource for researchers, students, and clinican-scientists interested in AAT deficiency, as well as anyone working in the fields of pulmonology and hepatology.

Editors and Affiliations

  • Horae Gene Therapy Center, University of Massachusetts Medical School, Worcester, USA

    Florie Borel, Christian Mueller

Bibliographic Information

  • Book Title: Alpha-1 Antitrypsin Deficiency

  • Book Subtitle: Methods and Protocols

  • Editors: Florie Borel, Christian Mueller

  • Series Title: Methods in Molecular Biology

  • DOI: https://doi.org/10.1007/978-1-4939-7163-3

  • Publisher: Humana New York, NY

  • eBook Packages: Springer Protocols

  • Copyright Information: Springer Science+Business Media LLC 2017

  • Hardcover ISBN: 978-1-4939-7161-9Published: 28 July 2017

  • Softcover ISBN: 978-1-4939-8403-9Published: 28 July 2018

  • eBook ISBN: 978-1-4939-7163-3Published: 26 July 2017

  • Series ISSN: 1064-3745

  • Series E-ISSN: 1940-6029

  • Edition Number: 1

  • Number of Pages: XIII, 278

  • Number of Illustrations: 29 b/w illustrations, 42 illustrations in colour

  • Topics: Molecular Medicine

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