Purine Metabolism in Man—II: Regulation of Pathways and Enzyme Defects

Front Matter....Pages i-xxii
History of Gout....Pages 1-12
Biochemical Studies of Purine Auxotrophs of Drosophila Melanogaster ....Pages 110-114
Inosine Triphosphate Metabolism in Human Erythrocytes....Pages 115-120
Oxypurine and 6-Thiopurine Nucleoside Triphosphate Formation in Human Erythrocytes....Pages 121-128
Activity of the Salvage-Pathway in Erythrocytes of Newborn Infants, Children and Adults....Pages 129-138
Purine Phosphoribosyl Transferases in Human Erythrocyte Ghosts....Pages 139-152
The Localisation of the Purine-Phosphoribosyltransferase in Rat Liver Organells....Pages 153-156
Reversible and Irreversible Inhibition of Hypo-Xanthine-Phosphoribosyltransferase....Pages 157-161
A Multienzyme Complex for De Novo Purine Biosynthesis....Pages 13-18
Coordinate Regulation of the Proximal and Distal Steps of the Pathway of Purine Synthesis De Novo in WI-L2 Human Lymphoblasts....Pages 19-29
Purine Toxicity in Human Lymphoblasts....Pages 30-39
Studies on the Regulation of the Biosynthesis of Myocardial Adenine Nucleotides....Pages 40-49
Effect of Triiodothyronine on the Biosynthesis of Adenine Nucleotides and Proteins in the Rat Heart....Pages 50-58
Altered Synthesis and Catabolism of Purine Nucleotides in Regulatory Mutants of Saccharomyces Cerevisiae ....Pages 59-63
Phosphoribosylpyrophosphate Synthesis in Human Erythrocytes: Inhibition by Purine Nucleosides....Pages 64-70
Human Phosphoribosylpyrophosphate Synthetase: Relation of Activity and Quaternary Structure....Pages 71-79
Transfer of Resistance to Selective Conditions from Fibroblasts with Mutant Feedback-Resistant Phosphoribosylpyrophosphate Synthetase to Normal Cells....Pages 80-84
Phosphoribosylpyrophosphate Degradation in Human Tissues....Pages 85-91
Properties of the Phosphoribosylpyrophosphate-Glutamine Amidotransferase of the Human Leucemic Cells....Pages 92-98
A Purine Auxotroph Deficient in Phosphoribosylpyrophosphate Amidotransferase and Phosphoribosylpyrophosphate Aminotransferase Activities with Normal Activity of Ribose-5-phosphate Aminotransferase....Pages 99-109
Purification and Characterization of Hypoxanthine-Guanine Phosphoribosyl Transferase from Cultured HTC Cells....Pages 162-171
Evidence for the Existence of Different Types of Metabolic Cooperation....Pages 172-180
Stability of the Azaguanine Resistant Phenotype In Vivo ....Pages 181-185
Functioning of Purine Salvage Pathways in Escherichia Coli K-12....Pages 186-195
Metabolism of Intravenous Adenine in the Pig....Pages 196-205
Pattern of Purine-Nucleotide Metabolism in Hepatopancreas of Helix Pomatia (Gastropoda)....Pages 206-211
Isozymes of AMP Deaminase....Pages 212-222
Purification of Human Erythrocyte Adenosine Deaminase....Pages 223-234
Adenosine Deaminase: Characterization of the Molecular Heterogeneity of the Enzyme in Human Tissue....Pages 235-248
Human 5′-Nucleotidase: Multiple Molecular Forms and Regulation....Pages 249-253
Subcellular distribution of purine degrading enzymes in the liver of the carp (Cyprinus carpio l.)....Pages 254-264
Purine Enzyme Abnormalities: A Four Year Experience....Pages 265-269
Fibroblast Phosphoribosylpyrophosphate and Ribose-5-Phosphate Concentration and Generation in Gout with Purine Overproduction....Pages 270-279
Familial Distribution of Increased Erythrocyte PP-Ribose-P Levels....Pages 280-286
X-Linked Pattern of Inheritance of Gout Due to Mutant Feedback-Resistant Phosphoribosylpyrophosphate Synthetase....Pages 287-292
Incidence of APRT Deficiency....Pages 293-294
Complete Deficiency of Adenine Phosphoribosyltransferase: Report of a Family....Pages 295-302
Purine Synthesis and Excretion in Mutants of the WI-L2 Human Lymphoblastoid Line Deficient in Adenosine Kinase (AK) and Adenine Phosphoribosyltransferase (APRT)....Pages 303-313
Partial HPRT Deficiency : Heterozygotes Exhibit One Cell Population in Intact Cell Assays....Pages 314-318
Diminished Affinity for Purine Substrates as a Basis for Gout with Mild Deficiency of Hypoxanthine-Guanine Phosphoribosyltransferase....Pages 319-325
Purine and Pyrimidine Nucleotide Concentrations in Cells with Decreased Hypoxanthine-Guanine-Phosphoribosyltransferase (HGPRT) Activity....Pages 326-340
Electrophoretic Variation in Partial Deficiencies of Hypoxanthine-Guanine Phosphoribosyltransferase....Pages 341-345
Xanthine Oxidase Activity in a Gouty Patient with Partial Deficiency of HGPRT....Pages 346-350
Experience with Detection of Heterozygous Carriers and Prenatal Diagnosis of Lesch-Nyhan Disease....Pages 351-358
HGPRT-Positive and HGPRT-Negative Erythrocytes in Heterozygotes for HGPRT Deficiency....Pages 359-360
Study of Immunoreactive Material in Patients with Deficient HPRT Activity....Pages 361-369
Effects of Inosine on Purine Synthesis in Normal and HGPRT-Deficient Human Fibroblasts....Pages 370-375
An Alternate Metabolic Route for the Synthesis of Inosine 5′-Phosphate (IMP) in the Lesch-Nyhan Erythrocyte....Pages 376-383
Adenosine Metabolism in Permanent Lymphocyte Lines and in Erythrocytes of Patients with the Lesch-Nyhan Syndrome....Pages 384-390
Purine-Nucleoside Phosphorylase and Adenosine Aminohydrolase Activities in Fibroblasts with the Leschnyhan Mutation....Pages 391-397
Altered Excretion of 5-Hydroxyindoleacetic Acid and Glycine in Patients with the Lesch-Nyhan Disease....Pages 398-404
Xanthinuria, Lithiasis and Gout in the Same Family....Pages 405-411
Immunological Aspects of Purine Metabolism....Pages 412-433
Phosphoribosylpyrophosphate (PRPP) Amidotransferase (EC 2.4.2.14) Activity in Unstimulated and Stimulated Human Lymphocytes....Pages 434-440
Adenosine and Guanosine Metabolism During Phytohemagglutinin Induced Transformation of Human Lymphocytes....Pages 441-447
Adenine and Adenosine Metabolism in Phytohemagglutinin (PHA) — Stimulated and Unstimulated Normal Human Lymphocytes....Pages 448-455
Adenine and Adenosine Metabolism in Lymphocytes Deficient in Adenosine Deaminase (ADA) Activity....Pages 456-462
Immunoreactive Adenosine Deaminase (ADA) in Cultured Fibroblasts from Patients with Combined Immunodeficiency Disease....Pages 463-470
Urinary Purines in a Patient with a Severely Defective T Cell Immunity and a Purine Nucleoside Phosphorylase Deficiency....Pages 471-476
A Second Case of Inosine Phosphorylase Deficiency with Severe T-Cell Abnormalities....Pages 477-480
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....Pages 481-487
Hepatic Gluconeogenesis and Urate Formation from Various Nucleosides....Pages 488-499
Influence of Carbohydrates on Uric Acid Synthesis in the Isolated Perfused Chicken Liver....Pages 500-508
Studies on the Influence of Ethanol and of Lactic Acid on Uric Acid Metabolism....Pages 509-518
Carbohydrate Induced Increase in Uric Acid Synthesis. Studies in Human Volunteers and in Laboratory Rats....Pages 519-528
Pharmacologically Induced Erythrocyte PP-Ribose-P Variations....Pages 529-534
Isolated Rat Liver Cells — Purine Metabolism and Effects of Fructose....Pages 535-541
Studies on the Effect of Fructose and Xylitol in the Rat Liver: 5′-Nucleotidase, Adenosine Deaminase, De Novo Purine Synthesis....Pages 542-552
The NADP/NADPH 2 Ratio in Erythrocytes of Hyperuricemic Patients, A Possible Regulator Between Purine, Carbohydrate and Lipid Metabolism....Pages 553-556
Hypertriglyceridaemia and Chenodeoxycholic Acid....Pages 557-562
Fatty Acid Composition of Plasma Lipid Fractions in Gout....Pages 563-569
An Improved Method for the Electrophoretic Separation and Histochemical Identification of 5-Phosphoribosyl-1-Pyrophosphate Synthetase Using Microgram Quantities of Crude Cell Extracts....Pages 570-574
A New Assay for the Determination of Phosphoribosylamine....Pages 575-585
Facilitated Purification of Hypoxanthine-Phosphoribosyltransferase by Affinity Chromatography....Pages 586-590
Ultramicrochemistry: A Contribution to the Analysis of Purine Metabolism in Man....Pages 591-596
Ultramicrochemical Studies on Enzyme Kinetics....Pages 597-604
Localization of Xanthine Oxidase Activity in Hepatic Tissue....Pages 605-609
Analysis of Purines and their Nucleosides by the Reverse Phase Partition Mode of High Pressure Liquid Chromatography....Pages 610-618
Enzymatic Determination of Urate, Cholesterol and Triglycerides in the Synovial Fluid with the Centrifugal Fast Analyzer....Pages 619-623
Back Matter....Pages 625-641