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|2 23
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|a Kalsheker, Noor,
|e author.
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1 |
0 |
|a Alpha-1-antitrypsin deficiency :
|b biology, diagnosis, clinical significance, and emerging therapies /
|c Noor Kalsheker, Robert Stockley.
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| 264 |
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1 |
|a London :
|b Academic Press, an imprint of Elsevier,
|c [2017]
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| 264 |
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|c �2017
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| 300 |
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|a 1 online resource
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| 336 |
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|a text
|b txt
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|a online resource
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| 504 |
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|a Includes bibliographical references and index.
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|a Vendor-supplied metadata.
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|a 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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|a 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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| 505 |
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|a 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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|a 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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|a 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
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| 650 |
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|a Alpha 1-antitrypsin deficiency.
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| 650 |
1 |
2 |
|a alpha 1-Antitrypsin Deficiency
|0 (DNLM)D019896
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| 650 |
2 |
2 |
|a Lung Diseases, Obstructive
|x etiology
|0 (DNLM)D008173Q000209
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| 650 |
2 |
2 |
|a Liver Diseases
|x etiology
|0 (DNLM)D008107Q000209
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| 650 |
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6 |
|a Carence en alpha-1-antitrypsine.
|0 (CaQQLa)201-0277949
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| 650 |
|
7 |
|a HEALTH & FITNESS
|x Diseases
|x General.
|2 bisacsh
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| 650 |
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7 |
|a MEDICAL
|x Clinical Medicine.
|2 bisacsh
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| 650 |
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7 |
|a MEDICAL
|x Diseases.
|2 bisacsh
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| 650 |
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7 |
|a MEDICAL
|x Evidence-Based Medicine.
|2 bisacsh
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| 650 |
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7 |
|a MEDICAL
|x Internal Medicine.
|2 bisacsh
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| 650 |
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7 |
|a Alpha 1-antitrypsin deficiency
|2 fast
|0 (OCoLC)fst00805916
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| 700 |
1 |
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|a Stockley, Robert A.,
|e author.
|
| 776 |
0 |
8 |
|i Print version:
|a Kalsheker, Noor.
|t Alpha-1-antitrypsin deficiency.
|d London : Academic Press, an imprint of Elsevier, [2017]
|z 0128039426
|z 9780128039427
|w (OCoLC)962889224
|
| 856 |
4 |
0 |
|u https://sciencedirect.uam.elogim.com/science/book/9780128039427
|z Texto completo
|
