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020 _a9781493971633
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024 7 _a10.1007/978-1-4939-7163-3
_2doi
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050 4 _aQH315-320
072 7 _aMBGR
_2bicssc
072 7 _aMED106000
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082 0 4 _a610.72
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245 1 0 _aAlpha-1 Antitrypsin Deficiency
_h[electronic resource] :
_bMethods and Protocols /
_cedited by Florie Borel, Christian Mueller.
250 _a1st ed. 2017.
264 1 _aNew York, NY :
_bSpringer New York :
_bImprint: Humana,
_c2017.
300 _aXIII, 278 p. 71 illus., 42 illus. in color.
_bonline resource.
336 _atext
_btxt
_2rdacontent
337 _acomputer
_bc
_2rdamedia
338 _aonline resource
_bcr
_2rdacarrier
347 _atext file
_bPDF
_2rda
490 1 _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.
650 0 _aMedicine
_xResearch.
650 0 _aBiology
_xResearch.
650 1 4 _aBiomedical Research.
700 1 _aBorel, Florie.
_eeditor.
_4edt
_4http://id.loc.gov/vocabulary/relators/edt
700 1 _aMueller, Christian.
_eeditor.
_4edt
_4http://id.loc.gov/vocabulary/relators/edt
710 2 _aSpringerLink (Online service)
773 0 _tSpringer Nature eBook
776 0 8 _iPrinted edition:
_z9781493971619
776 0 8 _iPrinted edition:
_z9781493971626
776 0 8 _iPrinted edition:
_z9781493984039
830 0 _aMethods in Molecular Biology,
_x1940-6029 ;
_v1639
856 4 0 _uhttps://doi.org/10.1007/978-1-4939-7163-3
912 _aZDB-2-PRO
999 _c6823
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