Contemporary Metabolism

Lieferzeit: Lieferbar innerhalb 14 Tagen

53,49 

Volume 1

ISBN: 1468434497
ISBN 13: 9781468434491
Herausgeber: Norbert Freinkel
Verlag: Springer Verlag GmbH
Umfang: xxvi, 498 S.
Erscheinungsdatum: 22.03.2012
Auflage: 1/2012
Produktform: Kartoniert
Einband: Kartoniert

Inhaltsangabe1 Disorders of Purine and Pyrimidine Metabolism.- 1.1. Introduction.- 1.2. Purine Metabolism.- 1.2.1. Hypoxanthine Reutilization as a Normal Regulator of Purine Synthesis de Novo.- 1.2.2. Drugs That Increase Purine Synthesis de Novo.- 1.2.3. Changes in Purine Metabolism with Cellular Proliferation.- 1.2.4. Role of the Purine Nucleotide Cycle.- 1.2.5. Role of the Purine Nucleotide Cycle in Transport Processes.- 1.2.6. Effect of Fructose.- 1.3. Adenosine Deaminase Deficiency Associated with Severe Combined Immunodeficiency Disease.- 1.3.1. Clinical Presentation.- 1.3.2. Frequency.- 1.3.3. Enzyme Abnormality.- 1.3.4. Screening Tests.- 1.3.5. Prenatal Diagnosis.- 1.3.6. Genetic Heterogeneity.- 1.3.7. Metabolic Studies.- 1.3.8. Model Systems of Adenosine Deaminase Deficiency.- 1.3.9. Treatment.- 1.4. Purine Nucleoside Phosphorylase Deficiency.- 1.4.1. Clinical Presentation.- 1.4.2. Enzyme Abnormalities.- 1.4.3. Purine Metabolites in Urine.- 1.4.4. Metabolic Studies.- 1.4.5. Treatment.- 1.4.6. Purine Nucleoside Phosphorylase Distribution in Human Tissues.- 1.5. Biochemical Basis of the Immunodeficiency in Adenosine Deaminase and Purkie Nucleoside Phosphorylase Deficiency.- 1.5.1. Ribonucleoside Accumulation.- 1.5.2. Pyrimidine Starvation.- 1.5.3. Possible Role of Cyclic AMP.- 1.5.4. Deoxyribonucleoside Accumulation.- 1.5.5. Implications for Therapy.- 1.6. Purine 5′-Nucleotidase Deficiency in Hypogammaglobulinemia.- 1.6.1. Clinical Categories with Low Purine 5′-Nucleotidase.- 1.6.2. Enzyme Characteristics.- 1.7. Adenine Phosphoribosyltransferase Deficiency.- 1.7.1. Clinical Presentation.- 1.7.2. Composition of Calculi.- 1.7.3. Purine Metabolites in the Urine and Plasma.- 1.7.4. Adenine Phosphoribosyltransferase Activity in Erythrocytes.- 1.7.5. Treatment.- 1.7.6. Metabolic Significance.- 1.7.7. Frequency.- 1.8. Hypoxanthine-Guanine Phosphoribosyltransferase Deficiency.- 1.8.1. Genetic Heterogeneity.- 1.8.2. Enzyme Characteristics.- 1.8.3. Enzyme Assays.- 1.8.4. Genetic Transformation.- 1.8.5. Neurological Disorders.- 1.8.6. Attempts to Produce a Hypoxanthine-Guanine- Phosphoribosyltransferase-Deficient Mouse.- 1.8.7. Mechanism of Excessive Purine Synthesis.- 1.8.8. Other Metabolic Correlations.- 1.9. Increased Phosphoribosyl-1-Pyrophosphate Synthetase Activity.- 1.9.1. Enzyme Characteristics.- 1.9.2. Inheritance.- 1.9.3. Biochemical Studies.- 1.10. Xanthinuria.- 1.10.1. Conditions Associated with Hypouricemia.- 1.10.2. Clinical Presentation.- 1.11. Gouty Arthritis.- 1.11.1. Hyperuricemia.- 1.11.2. Diagnosis.- 1.11.3. Disorders Associated with Gout.- 1.11.4. The Kidney and Gout.- 1.11.5. Association of Hyperuricemia and Vascular Disease.- 1.11.6. Association of Hyperuricemia with Avascular Necrosis of the Femoral Head.- 1.11.7. Acute Attack of Gout.- 1.11.8. Treatment.- 1.11.9. Metabolic Factors That Contribute to Hyperuricemia.- 1.12. Decreased Adenylate Deaminase Activity.- 1.13. Abnormalities of Pyrimidine Metabolism.- 1.13.1. Ammonia and Pyrimidine Nucleotide Synthesis.- 1.13.2. Pyrimidine 5′-Nucleotidase Deficiency.- 1.13.3. Orotic Aciduria.- 1.14. Abnormalities of DNA Repair (Xeroderma Pigmentosum).- 1.15. Purine and Pyrimidine Compounds as Inhibitors of Viral and Cellular Proliferation.- 1.15.1. Effective Clinical Treatment of Herpes Encephalitis with Adenine Arabinoside.- 1.15.2. New Antiviral Agents.- References.- 2 Metabolic Actions of Ethanol.- 2.1. Effects of Ethanol on Protein Metabolism.- 2.1.1. Origin of the Increased Liver Protein.- 2.1.2. Type of Proteins That Accumulate after Chronic Alcohol Consumption.- 2.1.3. Mechanisms of the Alcohol-Induced Accumulation of Liver Protein.- 2.1.3.1. Effects of Ethanol on Protein Synthesis.- 2.1.3.2. Effects of Ethanol on Protein Secretion.- 2.1.3.3. Effects of Ethanol on Protein Catabolism.- 2.1.4. Summary of the Alcohol-Induced Alterations of Hepatic Protein Metabolism and Possible Consequences.- 2.2. Effects of Ethanol on Lipid Metabolism.- 2.2.1. Liver Upids.- 2.2.2. Alcohol-In

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Beschreibung

Despite a new title, Contemporary Metabolism, Volume 1 is actually the third volume in a continuing series and succeeds The Year in Metabolism 1975- 1976 and The Year in Metabolism 1977. As in the earlier volumes, the same internationally recognized authorities review the noteworthy recent devel opments in their areas of expertise. In many instances they also address aspects that have not been considered previously. In this volume, Dr. J. Edwin Seegmiller again updates progress in understanding disorders of purine and pyrimidine metabolism. However, particular emphasis is placed on the emerging relationships with immune mechanisms. Dr. Charles S. Lieber is joined by Dr. Enrique Baraona in a continuing review of metabolic actions of ethanol. This chapter examines effects of ethanol on protein metabolism and selected features of lipid metabolism-two areas that were not included in the earlier volumes. Dr. DeWitt S. Goodman's review of disorders oflipid and lipoprotein metabo lism builds on his previous chapters, but much additional attention is directed to a critical analysis of recent advances in epidemiology and lipoprotein structures. In collaboration with Dr. Brian L. G. Morgan, Dr. Myron Winick devotes his entire chapter to a detailed review of the impact of nutrition upon brain development-an overview that has now been rendered possible by the burgeoning recent developments in this area.

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