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Blanco's overview of alpha-1 antitrypsin deficiency : history, biology, pathophysiology, related diseases, diagnosis and treatment /
| Clasificación: | Libro Electrónico |
|---|---|
| Autor principal: | |
| Formato: | Electrónico eBook |
| Idioma: | Inglés |
| Publicado: |
London, United Kingdom :
Academic Press is an imprint of Elsevier,
2017.
|
| Temas: | |
| Acceso en línea: | Texto completo |
Tabla de Contenidos:
- Cover; Title page; Copyright page; Dedication; Contents; Preface; Acknowledgments; Chapter 1
- Alpha-1 Antitrypsin Deficiency: Introduction and History; 1.1
- Introduction; 1.2
- History of the alpha-1 antitrypsin deficiency; References; Chapter 2
- Serpins and Serpinopathies; 2.1
- Introduction; 2.2
- The serpin superfamily; 2.2.1
- Classification and Nomenclature; 2.2.2
- Structure and Inhibitory Mechanisms; 2.2.3
- Polymerization; 2.2.4
- Serpinopathies; 2.2.4.1
- Alpha-1 Antitrypsin Deficiency; 2.2.4.2
- Alpha-1 Antichymotrypsin Deficiency; 2.2.4.3
- Antithrombin Deficiency
- 2.2.4.4
- C1 Inhibitor Deficiency2.2.4.5
- Neuroserpin Deficiency; 2.2.4.6
- Transcortin Deficiency; 2.2.4.7
- Thyroxine-Binding Globulin Deficiency; References; Chapter 3
- Alpha-1 Antitrypsin Biology; 3.1
- Structure; 3.2
- Synthesis and secretion; 3.3
- Substrate; 3.4
- Properties; 3.4.1
- Inhibition of Extracellular Serine Proteases; 3.4.2
- Inhibition of Transmembrane and Intracellular Proteases; 3.4.3
- Nonprotease-Inhibitory Antiinflammatory and Immunomodulatory Properties; 3.4.4
- Antimicrobial Properties; 3.4.5
- Protection and Repair of Damaged Tissues by Inflammation; References
- Chapter 4
- Alpha-1 Antitrypsin Gene, Genetic Heritage, Phenotypes, and Genotypes4.1
- The alpha-1 antitrypsin gene; 4.1.1
- Locus; 4.1.2
- Expression Regulation; 4.1.3
- Transcription Stimuli; 4.1.4
- AAT Transcription; 4.1.5
- Intracellular Mechanisms of Protein Folding and Secretion; 4.2
- The "protease inhibitor" system; 4.3
- Genetic inheritance; 4.4
- Nomenclature; References; Chapter 5
- Alpha-1 Antitrypsin Deficiency: Liver Pathophysiology; 5.1
- Introduction; 5.2
- The Z and S mutations; 5.3
- Pathophysiology; 5.4
- Clinical expression; References
- Chapter 6
- Alpha-1 Antitrypsin Deficiency: Lung Pathophysiology6.1
- Introduction; 6.2
- Physiopathological mechanisms of alpha-1 antitrypsin deficiency-related COPD; 6.2.1
- Alpha-1 Antitrypsin Genotypes Related to COPD Development; 6.2.2
- Mechanisms; 6.2.2.1
- Protease-Mediated Lung Disease; 6.2.2.2
- Oxidant-to-Antioxidant Imbalance; 6.2.2.3
- Tumor Necrosis Factor-Related Impaired Neutrophil Activity; 6.2.2.4
- Autoimmunity-Mediated COPD; 6.2.2.5
- Accumulation of Z Protein Polymers in the Airways; 6.3
- Clinical expression; References; Chapter 7
- Genetic Epidemiology
- 7.1
- Introduction7.2
- Pi*Z frequency and prevalence in EuropE; 7.2.1
- Origin and Spread of the Z Mutation; 7.3
- Pi*Z frequency in the world; 7.4
- Number and distribution of Pi*ZZ genotypes in Europe; 7.5
- Number and distribution of Pi*ZZ genotypes in America; 7.6
- Number and distribution of Pi*ZZ genotypes in Africa, Asia, Australia, and New Zealand; 7.7
- Pi*S frequency distribution in the world; 7.8
- Number and distribution of Pi*SZ genotypes in the world; References; Chapter 8
- Laboratory Diagnosis; 8.1
- Introduction
- 8.2
- Clinical situations in which alpha-1 antitrypsin deficiency should be suspected