Cargando…
Alpha-1-antitrypsin deficiency : biology, diagnosis, clinical significance, and emerging therapies /
| Clasificación: | Libro Electrónico |
|---|---|
| Autores principales: | , |
| Formato: | Electrónico eBook |
| Idioma: | Inglés |
| Publicado: |
London :
Academic Press, an imprint of Elsevier,
[2017]
|
| Temas: | |
| Acceso en línea: | Texto completo |
Tabla de Contenidos:
- 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
- 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
- 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
- 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
- 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