| Description |
1 online resource |
| Contents |
Cover ; Title page; Copyright page; Contents; List of Contributors; Preface; Acknowledgments; Chapter 1 -- Historical Perspective; Introduction; The discovery of alpha-1-antitrypsin deficiency (AATD); AATD and association with pulmonary emphysema; AATD and association with liver disease; Other disease associations; References; Further Reading; Chapter 2 -- The Swedish Alpha-1-Antitrypsin Screening Study: What We Have Learnt From Birth to Adult Life; Introduction; Fetal life; The liver; Infancy and Childhood; Adolescence and Young Adulthood; The Lungs; Smoking; Adaptive Biochemical Changes |
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Psychological Effects of the Neonatal Alpha-1-Antitrypsin ScreeningFinal Speculation; References; Chapter 3 -- Alpha-1-Antitrypsin Gene Regulation; Introduction; AAT, acute-phase, and tissue expression; The Serpin cluster on 14q31-31.2, the AAT gene, and transcripts; AAT hepatocyte promoter and enhancers; Control of AAT expression in nonhepatic tissue; AAT locus control region and interaction with transcription factors; References; Chapter 4 -- Alpha-1-Antitrypsin Variants; Introduction; Alpha-1-antitrypsin nomenclature and isoelectric focusing; Noncoding DNA variants; References |
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Further ReadingChapter 5 -- Alpha1-Antitrypsin: Structure and Dynamics in Health, Disease and Drug Development; Introduction; Why Understanding the Conformational Behavior of Alpha1-Antitrypsin Matters in Alpha1-Antitrypsin Deficiency; Conformational states associated with function and dysfunction; The Native Conformation; Complexed With Protease as a Consequence of the Functional Mechanism; How Structure Determines Function; The Cleaved Conformation; Finding of Polymers in Disease; Importance of dynamics; Dynamics of Native Wild-Type Alpha1-Antitrypsin in Solution |
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Alpha1-Antitrypsin Folding: How Does It Go Right, How Does It Go Wrong?How Does Alpha1-Antitrypsin Avoid Folding to the Inactive Latent Conformation?; Intracellular Misfolding and Polymerization of Alpha1-Antitrypsin; In vitro studies of Alpha1-Antitrypsin polymerization: lessons and limitations; Different Alpha1-Antitrypsin Polymers Form in Different Experimental Conditions; Studies of M*: the Polymerogenic Intermediate State; Disulfide Locking of Intracellular Polymers; Other Polymeric Architectures Observed In Vitro; Translation; Relating Deficiency Mutations to Disease Phenotype |
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Translating to New Therapeutic Strategies: Stabilizing Alpha1-Antitrypsin Against Pathological Conformational ChangeS4A Blockade; Allosteric stabilization; Polymerization Blockade by a Monoclonal Antibody; Blockade of Disease-Associated Dynamics; Studying polymerization in cells, organisms, and clinical samples; Conclusions; References; Chapter 6 -- Novel Biological Functions of A1AT; Introduction; A1AT is a broad-spectrum inhibitor of proteases; A1AT as an antiinflammatory and immunomodulatory protein; Modified molecular forms of A1AT and their biological functions; S-Nitrosylated A1AT |
| Bibliography |
Includes bibliographical references and index |
| Notes |
Vendor-supplied metadata |
| Subject |
Alpha 1-antitrypsin deficiency.
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alpha 1-Antitrypsin Deficiency
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Lung Diseases, Obstructive -- etiology
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Liver Diseases -- etiology
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HEALTH & FITNESS -- Diseases -- General.
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MEDICAL -- Clinical Medicine.
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MEDICAL -- Diseases.
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MEDICAL -- Evidence-Based Medicine.
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MEDICAL -- Internal Medicine.
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Alpha 1-antitrypsin deficiency
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| Form |
Electronic book
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| Author |
Stockley, Robert A., author.
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| LC no. |
2017446501 |
| ISBN |
9780128039472 |
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0128039477 |
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