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Main description:
The study of gouty arthritis has provided a common meeting ground for the research interests of both the basic scientist and the clinician. The interest of the chemist in gout began 1776 with the isolation of uric acid from a concretion of the urinary tract by the Swedish chemist SCHEELE. The same substance was subsequently extracted from a gouty tophus by the British chemist WOLLASTONE in 1797 and a half century later the cause of the deposits of sodium urate in such tophi was traced to a hyperuricemia in the serum of gouty patients by the British physician Alfred Baring GARROD who had also received training in the chemical laboratory and was therefore a fore-runner of many of today's clinician-investigators. The recent surge of progress in understanding of some of the causes of gout in terms of specific enzyme defects marks the entrance of the biochemist into this field of investigation. The identification of the first primary defect of purine metabolism associated with over-production of uric acid, a severe or partial deficiency of the enzyme hypoxanthine-guanine phospho ribosyltransferase was achieved less than a decade ago. The knowledge of the mechanism of purine over-production that it generated led shortly to the identification of families carrying a dominantly (possibly X-linked) inherited increase in the activity of the enzyme phosphoribosylpyrophosphate synthetase as a cause of purine over-production. Yet this is only a start as these two types of enzyme defects account for less than five per cent of gouty patients.
Contents:
of Volume 76 A.- History of Gout. Including Comments from an Illustrious Timeless Gathering.- Metabolic Pathways of Purines.- A Multienzyme Complex for De Novo Purine Biosynthesis.- Coordinate Regulation of the Proximal and Distal Steps of the Pathway of Purine Synthesis De Novo in WI-L2 Human Lymphoblasts.- Purine Toxicity in Human Lymphoblasts.- Studies on the Regulation of the Biosynthesis of Myocardial Adenine Nucleotides.- Effect of Triiodothyronine on the Biosynthesis of Adenine Nucleotides and Proteins in the Rat Heart.- Altered Synthesis and Catabolism of Purine Nucleotides in Regulatory Mutants of Saccharomyces Cerevisiae.- Phosphoribosylpyrophosphate Synthesis in Human Erythrocytes: Inhibition by Purine Nucleosides.- Human Phosphoribosylpyrophosphate Synthetase: Relation of Activity and Quaternary Structure.- Transfer of Resistance to Selective Conditions from Fibroblasts with Mutant Feedback-Resistant Phosphoribosylpyrophosphate Synthetase to Normal Cells. A Form of Metabolic Cooperation.- Phosphoribosylpyrophosphate Degradation in Human Tissues.- Properties of the Phosphoribosylpyrophosphate-Glutamine Amidotransferase of the Human Leucemic Cells.- A Purine Auxotroph Deficient in Phosphoribosylpyrophosphate Amidotransferase and Phosphoribosylpyrophosphate Aminotransferase Activities with normal Activity of Ribose-5-phosphate Aminotransferase.- Biochemical Studies of Purine Auxotrophs of Drosophila Melanogaster.- Inosine Triphosphate Metabolism in Human Erythrocytes.- Oxypurine and 6-Thiopurine Nucleoside Triphosphate Formation in Human Erythrocytes.- Activity of Salvage Pathway in Erythrocytes of Newborn Infants, Children, and Adults.- Purine Phosphoribosyltransferase in Human Erythrocyte Ghosts.- The Localisation of the Purine Phosphoribosyl-transferase in Rat Liver Organelles.- Reversible and Irreversible Inhibition of Hypoxanthine Phosphoribosyl-transferase.- Purification and Characterization of Hypoxanthine-Guanine Phosphoribosyltransferase from Cultured HTC Cells.- Evidence for the Existence of Different Types of Metabolic Cooperation.- Stability of the Azaguanine Resistant Phenotype in Vivo.- Functioning of Purine Salvage Pathways in Escherichia Coli K-12.- Metabolism of Intravenous Adenine in the Pig.- Pattern of Purine-Nucleotide Metabolism in Hepatopancreas of Helix Pomatia (Gastropoda).- Isozymes of AMP Deaminase.- Purification of Human Erythrocyte Adenosine Deaminase.- Adenosine Deaminase: Characterization of the Molecular Heterogeneity of the Enzyme in Human Tissue.- Human 5?-Nucleotidase : Multiple Molecular Forms and Regulation.- Subcellular Distribution of Purine Degrading Enzymes in the Liver of the Carp (Cyprinus Carpio L.).- Mutations Affecting Purine Metabolism in Man.- Purine Enzyme Abnormalities: A Four Year Experience.- Fibroblast Phosphoribosylpyrophosphate and Ribose-5-phosphate Concentration and Generation in Gout with Purine Overproduction.- Familial Distribution of Increased Erythrocyte Phosphoribosylpyrophosphate Levels.- X-linked Pattern of Inheritance of Gout due to Mutant Feedback-Resistant Phosphoribosylpyrophosphate Synthetase.- Incidence of APRT Deficiency.- Complete Deficiency of Adenine Phosphoribosyl-transferase: Report of a Family.- Purine Synthesis and Excretion in Mutants of the W1-L2 Human Lymphoblastoid Line Deficient in Adenosine Kinase (AK) and Adenine Phosphoribosyl-transferase (APRT).- Partial HPRT Deficiency: Heterozygotes Exhibit One Cell Population in Intact Cell Assays.- Diminished Affinity for Purine Substrates as a Basis for Gout with Mild Deficiency of Hypoxanthine-Guanine Phosphoribosyl-transferase.- Purine and Pyrimidine Nucleotide Concentrations in Cells with Decreased Hypoxanthine-Guanine Phosphoribosyltransferase (HGPRT) Activity.- Electrophoretic Variation in Partial Deficiencies of Hypoxanthine-Guanine Phosphoribosyltransferase.- Xanthine Oxidase Activity in a Gouty Patient with Partial Deficiency of HGPRT.- Experience with Detection of Heterozygous Carriers and Prenatal Diagnosis of Lesch-Nyhan Disease.- HGPRT-Positive and HGPRT-Negative Erythrocytes in Heterozygotes for HGPRT Deficiency.- Study of Immunoreactive Material in Patients with Deficient HPRT Activity.- Effects of Inosine on Purine Synthesis in Normal and HGPRT-Deficient Human Fibroblasts.- An Alternate Metabolic Route for the Synthesis of Inosine-5?-phosphate (IMP) in the Lesch-Nyhan Erythrocyte.- Adenosine Metabolism in Permanent Lymphocyte Lines and in Erythrocytes of Patients with the Lesch-Nyhan Syndrome.- Purine-Nucleoside Phosphorylase and Adenosine Aminohydrolase Activities in Fibroblasts with the Lesch-Nyhan Mutation.- Altered Excretion of 5-Hydroxyindoleacetic Acid and Glycine in Patients with the Lesch-Nyhan Disease.- Xanthinuria, Lithiasis and Gout in the Same Family.- Immunological Aspects.- Immunological Aspects of Purine Metabolism.- Phosphoribosylpyrophosphate Amidotransferase Activity in Unstimulated and Stimulated Human Lymphocytes.- Adenosine and Guanosine Metabolism During Phytohemagglutinin Induced Transformation of Human Lymphocytes.- Adenine and Adenosine Metabolism in Phytohemagglutinin-Stimulated and Unstimulated Normal Human Lymphocytes.- Adenine and Adenosine Metabolism in Lymphocytes Deficient in Adenosine Deaminase Activity.- Immunoreactive Adenosine Deaminase in Cultured Fibroblasts from Patients with Combined Immunodeficiency Disease.- Urinary Purines in a Patient with a Severely Defective T Cell Immunity and a Purine Nucleoside Phosphorylase Deficiency.- A Second Case of Inosine Phosphorylase Deficiency with Severe T-Cell Abnormalities.- Relationship Between Carbohydrate, Lipid and Purine Metabolism.- Regulation of De Novo Purine Synthesis in Human and Rat Tissue: Role of Oxidative Pentose Phosphate Pathway Activity and of Ribose-5-phosphate and Phosphoribosylpyrophosphate Availability.- Hepatic Gluconeogenesis and Urate Formation from Various Nucleosides.- Influence of Carbohydrates on Uric Acid Synthesis in the Isolated Perfused Chicken Liver.- Studies on the Influence of Ethanol and of Lactic Acid on Uric Acid Metabolism.- Carbohydrate Induced Increase in Uric Acid Synthesis. Studies in Human Volunteers and in Laboratory Rats.- Pharmacologically Induced Erythrocyte Phosphoribosylpyrophosphate Variations.- Isolated Rat Liver Cells - Purine Metabolism and Effects of Fructose.- Studies on the Effects of Fructose and Xylitol in the Rat Liver: 5?-Nucleotidase, Adenosine Deaminase, De Novo Purine Synthesis.- The NADP/NADPH2 Ratio in Erythrocytes of Hyperuricemic Patients, a Possible Regulator Between Purine, Carbohydrate and Lipid Metabolism.- Hypertriglyceridaemia and Chenodeoxycholic Acid.- Fatty Acid Composition of Plasma Lipid Fractions in Gout.- Methodology.- An Improved Method for the Electrophoretic Separation and Histochemical Identification of 5-Phosphoribosyl-1-pyrophosphate Synthetase Using Microgram Quantities of Crude Cell Extracts.- A New Assay for the Determination of Phosphoribosylamine.- Facilitated Purification of Hypoxanthine Phosphoribosyltransferase by Affinity Chromatography.- Ultramicrochemistry: A Contribution to the Analysis of Purine Metabolism in Man.- Ultramicrochemical Studies on Enzyme Kinetics.- Localization of Xanthine Oxidase Activity in Hepatic Tissue. A New Histochemical Method.- Analysis of Purines and their Nucleosides by the Reverse Phase Partition Mode of High Pressure Liquid Chromatography.- Enzymatic Determination of Urate, Cholesterol and Triglycerides in the Synovial Fluid with the Centrifugal Fast Analyzer.- Author Index.
PRODUCT DETAILS
Publisher: Springer (Springer-Verlag New York Inc.)
Publication date: December, 2012
Pages: 641
Weight: 1127g
Availability: Available
Subcategories: Biochemistry
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