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Physiology of Membrane Disorders -

Physiology of Membrane Disorders

Buch | Hardcover
1094 Seiten
1986 | 2nd edition
Kluwer Academic/Plenum Publishers (Verlag)
978-0-306-41774-0 (ISBN)
CHF 119,75 inkl. MwSt
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The second edition of Physiology of Membrane Disorders represents an extensive revision and a considerable expansion of the first edition . Yet the purpose ofthe second edition is identical to that of its predecessor, namely, to provide a rational analysis of membrane transport processes in individual membranes, cells, tissues, and organs, which in tum serves as a frame of reference for rationalizing disorders in which derangements of membrane transport processes playa cardinal role in the clinical expression of disease. As in the first edition, this book is divided into a number of individual, but closely related, sections. Part V represents a new section where the problem of transport across epithelia is treated in some detail. Finally, Part VI, which analyzes clinical derangements, has been enlarged appreciably. THE EDITORS xi Preface to the First Edition The purpose of this book is to provide the reader with a rational frame of reference for assessing the pa- thophysiology of those disorders in which derangements of membrane transport processes are a major factor responsible for the clinical manifestations of disease. In the present context, we use the term "membrane transport to refer to those molecular processes whose cardinal function, broadly speaking, is processes" in a catholic sense, the vectorial transfer of molecules-either individually or as ensembles-across biological interfaces, the latter including those interfaces which separate different intracellular compartments, the cellular and extracellular com- partments, and secreted fluids-such as glomerular filtrate-and extracellular fluids.

I: The Nature of Biological Membranes.- 1: The Anatomy of Biological Interfaces.- 1. Introduction.- 2. Models of Membrane Structure.- 3. Membrane Junctions.- 4. Certain Specialized Membranes.- 5. Lipid-Protein Interactions in Model Membranes.- 6. Membrane Fluidity.- 7. Mechanisms of Protein-Lipid Interactions.- 8. Mueller-Rudin Bilayer Membranes.- 9. Freeze-Fracture-Etch Studies of Membranes.- 10. Summary.- References.- 2: Composition and Dynamics of Lipids in Biomembranes.- 1. Molecular Organization of Lipids in Biomembranes.- 2. Lipid Composition of Mammalian Cell Membranes.- 3. Molecular Structure of Membrane Lipids.- 4. Molecular Motions in Bilayers.- 5. Interactions between Lipids in Bilayers.- 6. Summary.- References.- 3: Membrane Proteins: Structure, Arrangement, and Disposition in the Membrane.- 1. Introduction.- 2. Protein Composition of Membranes.- 3. Types of Membrane Proteins.- 4. Extrinsic Proteins.- 5. Intrinsic Proteins.- 6. Types of Intrinsic Membrane Proteins.- 7. The Structure of the Intramembrane Portion.- 8. Disposition of Proteins in the Membrane.- 9. Summary.- References.- II: Methods for Studying Membranes.- 4: The Nature and Limitations of Electron Microscopic Methods in Biology.- 1. Introduction.- 2. The Conventional Transmission Electron Microscope.- 3. Specimen Preparation.- 4. Embedding.- 5. Sectioning.- 6. Positive Staining.- 7. Negative Staining.- 8. Low-Dose Electron Microscopy.- 9. The Freeze-Fracture-Etch (FFE) Technique.- 10. Summary.- References.- 5: Isolation and Characterization of Biological Membranes.- 1. Introduction.- 2. Methods for Dissociating and Separating Cells.- 3. Isolation of Plasma Membranes.- 4. Assessment of Purification.- 5. Expression of Data.- 6. Functional Properties of Plasma Membranes.- 7. Pathological Considerations.- 8. Summary.- References.- 6: Absorption and Optical Rotation Spectra of Biological Membranes: Distortions and Their Corrections.- 1. Introduction.- 2. Distortions and Corrections for Suspensions.- 3. Purple Membrane as a Sample Calculation.- 4. Summary.- References.- 7: Mathematical Models of Membrane Transport Processes.- 1. Introduction.- 2. Diffusion.- 3. Osmosis.- 4. Ionic Diffusion.- 5. Facilitated Diffusion.- 6. Single-File Diffusion.- 7. Summary.- References.- 8: Application of Tracers to the Study of Membrane Transport Processes.- 1. Introduction.- 2. Tracers and Problems in the Use of Tracers.- 3. Isotope Effects.- 4. Rate Processes.- 5. Compartmental Systems and Compartmental Analysis.- 6. Theoretical Foundations for the Use of Tracers to Measure Rates.- 7. Applications to the Study of Membrane Transports.- 8. Summary.- References.- 9: Principles of Electrical Methods for Studying Membrane Movements of Ions.- 1. Introduction.- 2. Steady-State Characteristics of Biological Membranes.- 3. Non-Steady-State Electrical Properties of Membranes.- 4. Fluctuation Analysis of Electrical Properties of Membranes.- 5. Single-Channel Studies.- 6. Summary.- 7. Appendix.- References.- 10: The Study of Transport and Enzymatic Processes in Reconstituted Biological Systems.- 1. Introduction.- 2. Energy-Linked Processes.- 3. Receptors and Channels.- 4. Passive Transport System.- 5. Summary.- References.- III: General Problems in Membrane Biology.- 11: Principles of Water and Nonelectrolyte Transport across Membranes.- 1. Introduction.- 2. Diffusion as a Permeation Mechanism.- 3. Activation Energy for Diffusion: Measurement and Significance.- 4. Convection.- 5. Osmosis.- 6. Water and Nonelectrolyte Membrane Permeation Mechanisms.- 7. Summary.- References.- 12: Anion Transport in Erythrocytes.- 1. Introduction.- 2. Modes of Anion Transport.- 3. Anion Exchange in Human Erythrocytes.- 4. Net Anion Transport in Red Blood Cells.- 5. Cation-Coupled Anion Transport.- 6. Anion Transport in Other Single Cells.- 7. Relationship of Red Cell Anion Transport to Other Transport Systems.- 8. Summary.- References.- 13: Active Transport of Na+ and K+ by Red Blood Cells.- 1. Introduction.- 2. Intracellular Solutes and Water.- 3. Donnan and “Double Donnan” Equilibrium.- 4. Membrane Potential.- 5. Active Transport.- 6. Properties of the Na+,K+ Pump.- 7. Transport Modes of the Na+,K+ Pump.- 8. Ion Selectivity of the Na+,K+ Pump.- 9. Ouabain and the Na+,K+ Pump.- 10. Molecular Mechanism of the Na +,K + Pump.- References.- 14: Weak Electrolyte Transport across Biological Membranes: General Principles.- 1. Introduction.- 2. Weak Electrolyte Diffusion.- 3. Complex Mechanisms.- 4. Summary.- References.- 15: Ion Permeation in Cell Membranes.- 1. Introduction.- 2. How Ion Permeation Is Studied.- 3. Deviations from Independence.- 4. Gramicidin.- 5. Acetylcholine Receptor Channel.- 6. Voltage-Gated Sodium Channel.- 7. Potassium Channels.- 8. Calcium Channels.- 9. Summary.- References.- 16: Processing and Sorting of Proteins Synthesized in the Endoplasmic Reticulum.- 1. Introduction.- 2. Cotranslational Processing of Proteins.- 3. Posttranslational Processing of Proteins.- 4. Regulation of Protein Sorting and Membrane Interactions.- 5. Summary.- References.- 17: Ion-Coupled Transport of Organic Solutes across Biological Membranes.- 1. Introduction.- 2. Sodium-Coupled Sugar and Amino Acid Transport by Small Intestine and Renal Proximal Tubule.- 3. Proton-Coupled Galactoside Transport by E. coli.- 4. Toward a Mechanicokinetic Model of Ion-Coupled Nonelectrolyte Transport.- 5. Conclusions.- References.- 18: Bioenergetics of Membrane Transport Processes.- 1. Introduction.- 2. The Control of Mitochondrial Respiration.- 3. Thermogenesis.- 4. Respiration and Active Plasma Membrane Transport in Intact Cells.- 5. Summary.- References.- 19: Regulation of Cellular Volume.- 1. Introduction.- 2. Historical Perspective.- 3. The Gibbs-Donnan Equilibrium.- 4. The “Double Donnan” Hypothesis and Cellular Volume Regulation.- 5. Estimates of Cellular Colloid Osmotic Pressure.- 6. The Specificity of Cellular Sodium Chloride in Cellular Volume Regulation.- 7. Consequences of Metabolic Inhibition.- 8. Plasma Membrane Permeability and Cellular Volume.- 9. Regulation of Cellular Volume in Media Containing Cardiac Glycosides.- 10. Cellular Volume Regulation by Means Other Than the Cardiac Glycoside-Sensitive Na +, K + -ATPase.- 11. The Regulation of Volume in Cells Exposed to Anisosmotic Media.- 12. Summary and Conclusions.- References.- 20: Cell-to-Cell Communication: Permeability, Regulation, Formation, and Functions of the Cell-Cell Membrane Channel in Cell Junctions.- 1. Introduction.- 2. The Cell-to-Cell Membrane Channel.- 3. Regulation of the Cell-to-Cell Channel.- 4. Channel Formation.- 5. Physiological Roles of the Cell-to-Cell Channels.- References.- 21: Genes and Membranes.- 1. Introduction.- 2. Genetic Methods.- 3. Criteria for Establishing That Differences in Membrane Properties Are Genetically Determined.- 4. Information That Can Be Gained from Biochemical and Physiological Studies on Transport Mutants and from Cloning Studies.- 5. Information That Can Be Gained from Genetic Analysis.- 6. Summary.- References.- 22: The Interaction of Hormones with Biological Membranes.- 1. Introduction.- 2. Hormone Interaction with Receptor.- 3. Subunit Composition of Membrane Receptors.- 4. Transduction of Response to Interaction of Hormone and Receptor.- 5. Fate of Hormone-Receptor Complex.- 6. Down-Regulation of Surface Membrane Content of Receptors.- 7. Interactions between Receptors for Different Hormones.- 8. Summary.- References.- 23: Modification of Membrane Function by Drugs.- 1. Introduction.- 2. Drugs Affecting Transport Mechanisms of the Plasma Membrane.- 3. Drugs Affecting Excitation Phenomena.- 4. Drugs Affecting Coupling Mechanisms of Membranes.- 5. Summary.- References.- IV: Transport Events in Single Cells.- 24: Active Transport in Escherichia Coli: From Membrane to Molecule.- 1. Introduction.- 2. Membrane Vesicles and Active Transport: General Aspects.- 3. Energetics of Active Transport.- 4. Active Transport at the Molecular Level: The p-Galactoside Transport System.- 5. Summary.- References.- 25: Acidification of Intracellular Organelles: Mechanism and Function.- 1. Introduction.- 2. Evidence for Acid Interior.- 3. Generation of ?pH.- 4. Uses of ?pH.- 5. The Nature of the ATPase.- 6. Conclusion.- References.- 26: Intracellular pH Regulation.- 1. Introduction.- 2. Measurement of Intracellular pH.- 3. Cellular Buffering Processes.- 4. Effect of Externally Applied Weak Acids and Bases.- 5. Ion-Transport Systems.- References.- 27: Properties of Ionic Channels in Excitable Membranes.- 1. Introduction.- 2. How Do You Get a Resting Potential?.- 3. How Do You Change the Membrane Potential?.- 4. Ionic Channels.- 5. The Two-State Model.- 6. Real Channels Have More Than Two States.- 7. Na+ Channels.- 8. K+ Channels.- 9. Summary.- References.- 28: Ion Movements in Skeletal Muscle in Relation to the Activation of Contraction.- 1. Introduction.- 2. The Ultrastructure of the Tubular System in Skeletal Muscle Fibers.- 3. Electrical Properties of the Surface and Tubular Membrane.- 4. Inward Spread of Excitation.- 5. Cellular Ca2+ Movements Related to the Activation of Contraction.- 6. Summary.- References.- 29: Excitable Tissues: The Heart.- 1. Introduction.- 2. Multicellular Structure of the Heart.- 3. Electrical Activity in Different Regions of the Heart.- 4. Na + Channels and Excitability.- 5. Ca2+ Channels and Slow Responses.- 6. K+ Channels Support the Resting Potential and Action Potential Repolarization.- 7. Inward Currents and Pacemaker Activity.- 8. Adrenergic and Cholinergic Modulation of Cardiac Activity.- 9. Summary.- References.- 30: Ion Transport through Ligand-Gated Channels.- 1. Introduction and Overview.- 2. Structure of the Nicotinic AChR.- 3. Immunological Approaches to the Study of the Nicotinic AChR.- 4. Biogenesis, Membrane Localization, and Regulation.- 5. Dose-Response.- 6. Kinetics of Channel Gating.- 7. AChR Cation Selectivity and Permeation.- 8. Ligand-Gated Channels Other Than the AChR.- 9. An Emerging View of Transmitter-Activated Channels.- References.- V: Transport in Epithelia: Vectorial Transport through Parallel Arrays.- 31: Cellular Models of Epithelial Ion Transport.- 1. Introduction.- 2. Models of Sodium- and Chloride-Absorbing Epithelial Cells.- 3. A Model for Active Chloride Secretion by Epithelial Cells.- 4. Summary.- References.- 32: Ion Transport by Gastric Mucosa.- 1. Introduction.- 2. Organization of Gastric Epithelial Cells.- 3. Stimulus-Secretion Coupling in Oxyntic Cells.- 4. Metabolism and Energetics Associated with Gastric HC1 Secretion.- 5. Studies with Isolated Cell Fractions and Membranes.- 6. Electrophysiological and Tracer Flux Studies of Gastric Ion Transport.- 7. Summary.- References.- 33: Ion and Water Transport in the Intestine.- 1. Introduction.- 2. Models of Intestinal Na+, CI-, and H20 Transport.- 3. Intestinal Na + and Cl- Absorption.- 4. Intestinal Na+ and Cl- Secretion.- 5. HC03-, Short-Chain Fatty Acid, and K+ Transport.- 6. Shunt Pathway and Water Transport.- 7. Control of Intestinal Electrolyte Transport.- 8. Summary and Conclusions.- References.- 34: The Uptake of Lipids into the Intestinal Mucosa.- 1. Introduction.- 2. Chemical Species of Lipids That Are Involved during Fat Absorption.- 3. The Barriers to Lipid Absorption in the Intestine.- 4. Characteristics of the Intestinal Microvillus Membrane Barrier to Lipid Absorption.- 5. Characteristics of the Intestinal Unstirred Water Layer Barrier to Lipid Absorption.- 6. Characteristics of Fatty Acid and Cholesterol Absorption in the Intestine.- 7. Role of Bile Acid Micelles in Facilitating Lipid Absorption in the Intestine.- 8. Nonpolar Lipids.- 9. Summary Description of the Process of Lipid Uptake.- References.- 35: Mechanisms of Bile Secretion and Hepatic Transport.- 1. Introduction.- 2. Structural Determinants of Bile Secretory Function.- 3. Mechanisms of Hepatocellular Water and Electrolyte Secretion.- 4. Other Primary Driving Forces for Canalicular Bile Secretion (Bile Acid- Independent Secretion).- 5. Model for Hepatocyte Water and Electrolyte Secretion.- 6. Physiological Modifiers of Hepatocyte Bile Formation.- 7. Organic Anion Solute Transport.- 8. Lipid Excretion in Bile.- 9. Proteins in Bile.- 10. Miscellaneous Substances Found in Bile.- 11. Bile Duct Function.- 12. Summary.- References.- 36: The Regulation of Glomerular Filtration Rate in Mammalian Kidneys.- 1. Introduction.- 2. Ultrastructural Considerations.- 3. Characteristics of the Filtration Process.- 4. Quantitative Description of Glomerular Dynamics.- 5. Physiological Regulation of Glomerular Filtration Rate.- 6. Intrarenal Distribution of Glomerular Filtration Rate.- 7. Summary.- References.- 37: The Proximal Nephron.- 1. General Properties of the Proximal Nephron.- 2. Distribution of Transport Functions along the Proximal Tubule.- 3. Transepithelial Potentials and Passive Permeabilities.- 4. NaCl and NaHC03 Transport.- 5. Solute-Solvent Coupling—Role of the Intercellular Shunt Pathway.- References.- 38: The Effects of ADH on Salt and Water Transport in the Mammalian Nephron: The Collecting Duct and Thick Ascending Limb of Henle.- 1. Introduction.- 2. Intracellular Mediators of ADH Action.- 3. The Medullary Thick Ascending Limb.- 4. The Collecting Tubule.- 5. Homology of Hormone Action.- 6. Modulation of the ADH Response.- 7. Summary: Integration of ADH Action on Urinary Concentration.- References.- 39: Urinary Concentrating and Diluting Processes.- 1. Introduction.- 2. Renal Structure.- 3. Basic Concepts.- 4. Handling of Individual Solutes in the Medulla.- 5. Properties of the Thin Limbs of Henle’s Loops.- 6. Concentration in the Inner Medulla.- 7. Summary.- References.- 40: Transport Functions of the Distal Convoluted Tubule.- 1. Introduction.- 2. Structural Heterogeneity.- 3. Transepithelial Net Transport of Solutes and Water.- 4. Electrophysiological Considerations.- 5. Mechanisms of Transport.- 6. Summary.- References.- 41: The Respiratory Epithelium.- 1. Introduction.- 2. The Tracheal Epithelium.- 3. The Bronchial Epithelium.- 4. The Alveolar Epithelium.- 5. The Fetal Lung.- 6. Summary.- References.- VI: Clinical Disorders of Membrane Transport Processes.- 42: The Cellular Basis of Ischemic Acute Renal Failure.- 1. Introduction.- 2. Ischemic Injury.- 3. Summary.- References.- 43: Genetic Variants Affecting the Structure and Function of the Human Red Cell Membrane.- 1. Introduction.- 2. Intrinsic Membrane Abnormalities.- 3. Hemoglobinopathies.- 4. Endocrine Disorders.- 5. Manic-Depressive Disease.- 6. Essential Hypertension.- 7. Inherited Neuromuscular Disorders.- 8. Adenosine Deaminase Variants.- References.- 44: Inherited Membrane Disorders of Muscle: Duchenne Muscular Dystrophy and Myotonic Muscular Dystrophy.- 1. Introduction.- 2. Myotonic Muscular Dystrophy.- 3. Duchenne Muscular Dystrophy.- 4. Summary.- References.- 45: Disorders of Muscle: The Periodic Paralyses.- 1. Introduction.- 2. Clinical Features.- 3. Pathophysiology of the Periodic Paralysis.- 4. Therapy and Its Rationale.- 5. Summary.- References.- 46: Pathophysiology of Cardiac Arrhythmias.- 1. Introduction.- 2. Abnormalities of Impulse Propagation.- 3. Interactions between Abnormal Impulse Formation and Propagation.- 4. Electrophysiological Mechanisms Responsible for Clinically Occurring Arrhythmias.- 5. Summary.- References.- 47: Pathophysiology of Peptic Ulcer Disease.- 1. Introduction.- 2. Gastric vs. Duodenal Ulcer.- 3. Acid-Pepsin Secretion.- 4. Mucosal Resistance.- 5. Conclusions.- References.- 48: Malabsorption Syndromes.- 1. Introduction.- 2. Lipid Digestion and Absorption.- 3. Normal Enterohepatic Circulation of Bile Acids.- 4. Carbohydrate and Protein Digestion and Absorption.- 5. Tests of Intestinal Digestive and Absorptive Function.- 6. Diseases Affecting Normal Digestion or Absorption.- 7. Summary.- References.- 49: Pathophysiology of Calcium Absorptive Disorders.- 1. Introduction.- 2. Regulation of Intestinal Calcium Absorption.- 3. Sites of Calcium Absorption.- 4. Mechanism of Calcium Absorption.- 5. The Role of Vitamin D.- 6. Clinical Disorders Associated with Alterations in Calcium Absorption.- 7. Increased Absorption of Calcium.- 8. Malabsorption of Calcium.- 9. Drug-Induced Inhibition of Calcium Absorption.- 10. Nutritional Factors.- References.- 50: Cystic Fibrosis.- 1. Introduction.- 2. Clinical Features.- 3. Prognosis.- 4. Genetics.- 5. Pathogenesis.- 6. Animal Models.- 7. Commentary.- 8. Summary.- References.- 51: Disorders of Glomerular Filtration.- 1. Introduction.- 2. Some General Truths.- 3. Some General Observations on the Mechanism of Change in GFR in the Physiologic Setting.- 4. Potential Interactions among the Determinants of Glomerular Ultrafiltration.- 5. The Influence of Humoral and Hormonal Substances upon Glomerular Ultrafiltration.- 6. Angiotensin II.- 7. The Adrenergic Nervous System.- 8. ADH, cAMP.- 9. Prostaglandins, Thromboxanes, Kinins.- 10. PTH, Ca2+.- 11. Papaverine, Acetylcholine, Histamine, Methylprednisolone.- 12. The Final Common Pathway Viewpoint.- 13. Glomerular Ultrafiltration in Altered Physiologic States.- 14. Influences of Tubuloglomerular Feedback System on the Process of Glomerular Filtration.- 15. Pathophysiologic Conditions.- 16. Glomerular Immune Injury.- 17. Glomerular Effects of Systemic Hypertension.- 18. Isolated Glomeruli in Vitro.- 19. Causes of Progressive Reduction in GFR.- 20. Summary.- References.- 52: The Hypertonic and Hypotonic Syndromes.- 1. Introduction.- 2. Antidiuretic Hormone.- 3. Thirst.- 4. The Hypertonic Syndromes.- 5. The Hypotonie Syndromes.- 6. Summary.- References.- 53: Disorders of Proton Secretion by the Kidney.- 1. Introduction.- 2. Proton Transport across Urinary Epithelia.- 3. Functional Organization of H+ Secretion in the Kidney.- 4. The Renal Acidoses.- 5. Reduced Nephron Population.- 6. Renal Hypoperfusion.- 7. Renal Tubular Acidosis.- 8. Proximal Renal Tubular Acidosis.- 9. Distal Renal Tubular Acidosis.- 10. Impaired Excretion of Net Acid and Potassium (Normal pH Gradient Formation).- 11. Clinical Aspects of Renal Tubular Acidosis and Associated Disorders of Electrolyte Transport.- 12. Summary.- References.- 54: Cystinosis and the Fanconi Syndrome.- 1. Introduction.- 2. Historical Resume.- 3. Clinical and Pathological Features.- 4. Chemistry and Metabolism of Cystine.- 5. Other Causes of the Fanconi Syndrome.- 6. Treatment.- 7. Summary.- References.- 55: Renal Tubular Defects in Phosphate and Amino Acid Transport.- 1. Introduction.- 2. Defects in Phosphate Transport Processes.- 3. Aminoacidurias.- 4. Summary.- References.- 56: Pulmonary Edema.- 1. Introduction.- 2. Starling Force Analysis.- 3. Safety Factors Associated with Hydrostatic Edemas.- 4. Safety Factors Associated with Noncardiac Edema.- 5. Mechanisms of Intraalveolar Edema Formation.- 6. Summary.- References.

Erscheint lt. Verlag 31.1.1986
Zusatzinfo 383 Illustrations, black and white; 1094 p. 383 illus.
Verlagsort New York
Sprache englisch
Gewicht 2780 g
Themenwelt Medizinische Fachgebiete Innere Medizin Nephrologie
ISBN-10 0-306-41774-X / 030641774X
ISBN-13 978-0-306-41774-0 / 9780306417740
Zustand Neuware
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