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Title Alpha-1 antitrypsin deficiency : methods and protocols / edited by Florie Borel, Christian Mueller
Published New York : Humana Press : Springer, ©2017

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Description 1 online resource (xiii, 278 pages) : illustrations (some color)
Series Methods in molecular biology, 1940-6029 ; volume no. 1639
Methods in molecular biology (Clifton, N.J.) ; v. 1639. 1064-3745
Contents Pathophysiology of alpha-1 antitrypsin deficiency liver disease / Jeffrey H. Teckman and Keith S. Blomenkamp -- Pathophysiology of alpha-1 antitrypsin lung disease / Michael Kalfopoulos, Kaitlyn Wetmore, and Mai K. ElMallah -- Measuring and interpreting serum AAT concentration / Leslie J. Donato, Melissa R. Snyder, and Dina N. Greene -- AAT phenotype identification by isoelectric focusing / Dina N. Greene, M.C. Elliott-Jelf, and David G. Grenache -- Laboratory diagnosis by genotyping / Irene Belmonte, Luciana Montoto, and Francisco Rodrıguez-Frıas -- Genotyping protocol for the alpha-1 antitrypsin (PiZ) mouse model / Alisha M. Gruntman -- Elastase-induced lung emphysema models in mice / Bela Suki, Erzsebet Bartolak-Suki, and Patricia R.M. Rocco -- Assessing structure-function relations in mice using the forced oscillation technique and quantitative histology / Harikrishnan Parameswaran and Bela Suki -- 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, and Andrew M. Hoffman -- Design, cloning, and in vitro screening of artificial miRNAs to silence alpha-1 antitrypsin / Florie Borel and Christian Mueller -- Methods to identify and characterize siRNAs targeting serpin A1 in vitro and in vivo using RNA interference / Stuart Milstein, Kun Qian, Linying Zhang, and Alfica Sehgal -- Knockdown of Z mutant alpha-1 antitrypsin in vivo using modified DNA antisense oligonucleotides / Mariam Aghajan, Shuling Guo, and Brett P. Monia -- Immunohistochemistry staining for human alpha-1 antitrypsin / Dongtao A. Fu and Martha Campbell-Thompson-- Periodic acid-schiff staining with diastase / Dongtao A. Fu and Martha Campbell-Thompson -- Protocol for directed differentiation of human induced pluripotent stem cells (iPSCs) to a hepatic lineage / Joseph E. Kaserman and Andrew A. Wilson -- Isolation of kupffer cells and hepatocytes from a single mouse liver / Marcela Aparicio-Vergara, Michaela Tencerova, Cecilia Morgantini, Emelie Barreby, and Myriam Aouadi -- Alpha-1 antitrypsin transcytosis and secretion / Angelia D. Lockett -- Measuring the effect of histone deacetylase inhibitors (HDACi) on the secretion and activity of alpha-1 antitrypsin / Chao Wang, Marion Bouchecareilh, and William E. Balch -- Expression and purification of active recombinant human Alpha-1 antitrypsin (AAT) from Escherichia coli / Beena Krishnan, Lizbeth Hedstrom, Daniel N. Hebert, Lila M. Gierasch, and Anne Gershenson -- Quantification of total human alpha-1 antitrypsin by sandwich ELISA / Qiushi Tang, Alisha M. Gruntman, and Terence R. Flotte -- Quantification of murine AAT by direct ELISA / Andrew Cox and Christian Mueller -- Quantification of Z-AAT by a Z-specific "sandwich" ELISA / Florie Borel, Qiushi Tang, and Christian Mueller -- Semiquantitation of monomer and polymer alpha-1 antitrypsin by centrifugal separation and assay by western blot of soluble and insoluble components / Keith S. Blomenkamp and Jeffrey H. Teckman -- Electrophoresis- and FRET-based measures of serpin polymerization / Sarah V. Faull, Anwen E. Brown, Imran Haq, and James A. Irving -- Therapeutics : alpha-1 antitrypsin augmentation therapy / Michael Campos and Jorge Lascano -- Therapeutic options in alpha-1 antitrypsin deficiency : liver transplantation / Nedim Hadzic -- Therapeutics : gene therapy for alpha-1 antitrypsin deficiency / Alisha M. Gruntman and Terence R. Flotte
Summary 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
Bibliography Includes bibliographical references and index
Notes Online resource; title from PDF title page (SpringerProtocol, viewed July 31, 2017)
Subject Alpha 1-antitrypsin deficiency -- Laboratory manuals
Alpha 1-antitrypsin deficiency.
alpha 1-Antitrypsin Deficiency
Models, Animal
Vitro Techniques
Medical research.
Science -- Life Sciences -- Biology -- Molecular Biology.
Alpha 1-antitrypsin deficiency
Genre/Form Laboratory manuals
Laboratory manuals.
Manuels de laboratoire.
Form Electronic book
Author Borel, Florie, editor
Mueller, Christian, editor
ISBN 9781493971633
1493971638
1493971611
9781493971619