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008			170727s2017 xxu| s |||| 0|eng d
020			_a9781493971633
024	7		_a10.1007/978-1-4939-7163-3 _2doi
040			_aES-MaUEC _bspa _cES-MaUEC
245	1	0	_aAlpha-1 Antitrypsin Deficiency _bMethods and Protocols _cedited by Florie Borel, Christian Mueller.
250			_a1st edition 2017
264		1	_aNew York, NY _bSpringer International Publishing _c2017
300			_a1 recurso en línea (XIII, 278 páginas) _b71 ilustraciones, 42 ilustraciones a color
336			_atexto _btxt _2rdacontent
337			_aelectrónico _bc _2rdamedia
338			_arecurso electrónico _bcr _2rdacarrier
347			_aarchivo de texto _bPDF
490	0		_aMethods in Molecular Biology _x1940-6029 _v1639
505	0		_aPathophysiology of Alpha-1 Antitrypsin Deficiency Liver Disease -- Pathophysiology of Alpha-1 Antitrypsin Lung Disease -- Measuring and Interpreting Serum AAT Concentration -- AAT Phenotype Identification by Isoelectric Focusing -- Laboratory Diagnosis by Genotyping -- Genotyping Protocol for the Alpha-1 Antitrypsin (PiZ) Mouse Model -- Elastase-Induced Lung Emphysema Models in Mice -- Assessing Structure-Function Relations in Mice Using the Forced Oscillation Technique and Quantitative Histology -- 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 -- Design, Cloning, and In Vitro Screening of Artificial miRNAs to Silence Alpha-1-Antitrypsin -- Methods to Identify and Characterize siRNAs Targeting Serpin A1 In Vitro and In Vivo using RNA Interference -- Knockdown of Z Mutant Alpha-1 Antitrypsin in Vivo Using Modified DNA Antisense Oligonucleotides -- Immunohistochemistry Staining for Human Alpha-1 Antitrypsin -- Periodic Acid-Schiff Staining with Diastase -- Protocol for Directed Differentiation of Human Induced Pluripotent Stem Cells (iPSCs) to a Hepatic Lineage -- Isolation of Kupffer Cells and Hepatocytes from a Single Mouse Liver -- Alpha-1 Antitrypsin Transcytosis and Secretion -- Measuring the Effect of Histone Deacetylase Inhibitors (HDACi) on the Secretion and Activity of Alpha-1 Antitrypsin -- Expression and Purification of Active Recombinant Human 1-Antitrypsin (AAT) from Escherichia coli -- Quantification of Total Human Alpha-1 Antitrypsin by Sandwich ELISA -- Quantification of Murine AAT by Direct ELISA -- Quantification of Z-AAT by a Z-Specific "Sandwich" ELISA -- Semi-Quantitation of Monomer and Polymer Alpha-1 Antitrypsin by Centrifugal Separation and Assay by Western Blot of Soluble and Insoluble Components -- Electrophoresis- and FRET-Based Measures of Serpin Olymerization -- Therapeutics: Alpha-1 Antitrypsin Augmentation Therapy -- Therapeutic Option in Alpha-1 Antitrypsin Liver Transplantation -- Therapeutics: Gene Therapy for Alpha-1 Antitrypsin Deficiency.
520			_aThis 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.
700	1		_aBorel, Florie _eeditor literario _4edt _4http://id.loc.gov/vocabulary/relators/edt
700	1		_aMueller, Christian _eeditor literario _4edt _4http://id.loc.gov/vocabulary/relators/edt
776	0	8	_iPrinted edition: _z9781493971619
776	0	8	_iPrinted edition: _z9781493971626
776	0	8	_iPrinted edition: _z9781493984039
856	4	0	_uhttps://go.openathens.net/redirector/universidadeuropea.es?url=https://doi.org/10.1007/978-1-4939-7163-3 _z(usuarios Universidad Europea de Valencia)
942			_2lcc _cLE
988			_aSpringer_Protocols_2017
999			_c234180 _d234180