Molecular Basis of Membrane-Associated Diseases

ISBN-13: 9783642744174 / Angielski / Miękka / 2011 / 435 str.

Angelo Azzi; Zdenek Drahota; Sergio Papa

Opis

Biological membranes are often effected by diseases. Molecular events leading to or arising from pathological changes in the course of different diseases are as yet not clearly understood. This competent study by leading experts covers changes of the cellular environment, membranes and the metabolic functions during tissue growth and differentiation as well as aspects of abnormal organelle function in lysosomal storage diseases, peroxisomal and mitochondrial disorders, enzyme defects and regulatory defects of receptors due to oncogenes.

Spis treści

1 Plasma Membrane and Control of Ceü Growth and Differentiation.- Insulin Receptor Kinase and Insulin Action.- Structural and Functional Aspects of Signal Transduction by Receptor Tyrosine Kinases.- Nerve Growth Factor (NGF): Physiological Functions and Regulation of Its Synthesis.- Signal Transduction by Growth Factor Receptors.- Altered Regulation of Receptor-Associated Tyrosine Kinases in Human Tumors.- The Role of Plasma Membrane Phospholipids in Cellular Pathology.- Changes in the Structure and Function of the Red Cell Membrane Skeleton and Hemolytic Anemias.- Regulation of Intracellular Ca2+ in Pancreatic Acinar Cells by Membrane Pumps and Channels.- Control of Protein Kinase C Function by Activators and Inhibitors.- 2 Regulation of Membrane Channels.- K+ Channels: Structure, Function, Regulation, Molecular Pharmacology and Role in Diseased States.- Voltage-Dependent Calcium Channels: Structure and Regulation in Normal and Abnormal States.- Cell Membrane Abnormalities in Genetic Hypertension.- Transmembrane Cation Transport: An Approach to the Study of the Molecular Basis of Hypertension.- Transmembrane Ionic Fluxes in the Brain in Energy-Depleted Cells.- 3 Mitochondrial Genetic Diseases.- Mitochondrial Diseases.- Mitochondrial Myopathy: Biochemical Approaches to Respiratory Chain-Linked Electron Transfer and Energy Coupling.- Phosphorus Magnetic Resonance Spectroscopy (31P NMR) as a Tool for in Vivo Monitoring of Mitochondrial Muscle Disorders.- Cytochemical and Immunocytochemical Studies of Human Mitochondrial Myopathies.- Regulatory Complexity of Cytochrome c Oxidase and Its Defective Manifestation in Mitochondrial Diseases.- Fibroblasts and Cytochrome c Oxidase Deficiency.- Human Cytochrome c Oxidase Deficiencies; Structural and Functional Aspects.- 4 Mitochondrial Metabolic Diseases.- Brown Adipose Tissue, Diet-Induced Thermogenesis and Genetic Obesity.- Acute and Long-Term Regulation of Brown Adipose Tissue Thermogenesis: Physiological and Pathological Implications.- Molecular Composition, Fluidity of Membranes and Functional Properties of Human Liver Mitochondria and Microsomes.- Changes in Erythrocyte and Platelet Membrane Fluidity in Childhood Obesity.- Skeletal Muscle Mitochondria and Phospholipase Activity in Malignant Hyperthermia.- The Antimitochondrial Antibodies (AMA) of Primary Biliary Cirrhosis (PBC).- Toxicity, Antioxidants, and Metabolism.- 5 Cancer and Energy Metabolism.- Mitochondrial H+-ATP Synthase Under Normal and Pathological Conditions.- Antitumor Effect of Drugs Interfering with Mitochondrial Biogenesis.- 6 Lysosomes and Peroxisomes in Health and Disease.- The Complex of ²-Galactosidase, Neuraminidase and “Protective Protein” in Lysosomes: Molecular Characterization of the “Protective Protein”.- The Role of Activator Proteins in Glycolipid Degradation and Storage Diseases.- Solute Translocation Across the Lysosome Membrane: Physiology, Pathology and Pharmacology.- The Peroxisomal ?-Oxidation Systems: Characteristics and (Dys) Functions in Man.- Genetic Heterogeneity in Inherited Disorders with a Generalized Impairment of Peroxisomal Functions: Visualization by Immunofluorescence Microscopy of Peroxisome Assembly After Somatic Cell Fusion of Complementary Cell Lines.