Purine Metabolism in Man-IV: Part A: Clinical and Therapeutic Aspects; Regulatory Mechanisms

Front Matter....Pages i-xvii
Diagnostic and Therapeutic Approaches in Pyrimidine 5′-Nucleotidase Deficiency....Pages 103-108
New Defects of Pyrimidine Metabolism....Pages 109-114
Lymphocyte Ecto 5′-Nucleotidase in Immunodeficiency and Leukaemia....Pages 67-72
Clinicobiochemical Analysis of Four Cases of Xanthine Oxidase Deficiency....Pages 73-76
Human Myoadenylate Deaminase Deficiency....Pages 77-84
Myoadenylate Deaminase Deficiency: An Enzyme Defect in Search of a Disease....Pages 85-89
Problems in Diagnosis and Treatment of Adenine and Hypoxanthine-Guanine Phosphoribosyltransferase Deficiency....Pages 1-6
Problems of Diagnosis in an Adolescent with Hypoxanthine-Guanine Phosphoribosyltransferase Deficiency and Acute Renal Failure....Pages 7-12
Hypoxanthine-Guanine Phosphoribosyl Transferase (HGPRT) Deficiency in a Girl....Pages 13-18
Clinical and Biochemical Correlates of A New HPRT Mutation....Pages 19-22
Enzyme Replacement in the Lesch-Nyhan Syndrome with Long-Term Erythrocyte Transfusions....Pages 23-26
Adeninephosphoribosyltransferase (APRT)-Activity in Patients with Nephrolithiasis or Renal Failure....Pages 27-31
New Perspectives in the Diagnosis and Treatment of Adenosine Deaminase (ADA) Deficiency....Pages 33-38
Intravenous Deoxycytidine Therapy in a Patient with Adenosine Deaminase Deficiency....Pages 39-45
Properties of a Novel PEG Derivative of Calf Adenosine Deaminase....Pages 47-52
Phosphoribosylpyrophosphate Synthetase Superactivity: Detection, Characterization of Underlying Defects, and Treatment....Pages 91-96
Evidence of A New Syndrome Involving Hereditary Uric Acid Overproduction, Neurological Complications and Deafness....Pages 97-102
Purine Nucleoside Phosphorylase (PNP) Deficiency: A Therapeutic Challenge....Pages 53-59
The Effect of Deoxycytidine and Tetrahydrouridine in Purine Nucleoside Phosphorylase Deficiency....Pages 61-66
Clinical Survey of 200 Patients with Gout....Pages 115-118
Incorporation of 15 N from Glycine into Uric Acid in Gout: A Follow-Up Study....Pages 119-121
Hyperuricaemia in Young New Zealand Maori Men....Pages 123-128
Lean Dry Gout Patients....Pages 129-132
Urate Deposits without Gouty Arthritis....Pages 133-136
Postheparin Plasma Lipoprotein Lipase and Hepatic Triglyceride Lipase Activities in Gout....Pages 137-139
HDL-Cholesterol Levels in Gouty Patients....Pages 141-144
Urinary cAMP Excretion in Gouty Patients with and Without Nephrolithiasis....Pages 145-149
Hyperuricemia in Primary Hyperparathyroidism: Incidence and Evolution after Surgery....Pages 151-157
Pilot Study of Blood Coagulation in Gout Patients....Pages 159-162
Characterization of an Urate Binding α 2 -Globulin from Human Serum....Pages 163-166
Metabolic Studies of High Doses of Allopurinol in Humans....Pages 167-170
Gout Resistant to Allopurinol: Poor Compliance or Non-Response....Pages 171-174
Uricostatic Effect of Allopurinol in the Allantoxanamide-Treated Rat: A New Method for Evaluating Antiuricopathic Drugs....Pages 175-178
The Excretion of 14 C-Hypoxanthine and Its Metabolites in Rats Following Administration of Uricostatic Drugs....Pages 179-183
Pulse Radiolysis Study of Hypoxanthine and Xanthine Oxidation....Pages 185-188
Urate Crystal-Induced Superoxide Radical Production by Human Neutrophils....Pages 189-192
Renal Handling of Uric Acid in Normal Subjects: Its Behaviour with Respect to Different Filtered Loads....Pages 193-195
Evidence of Abnormal Renal Handling of Uric Acid in Patients with Nephrolithiasis and Hyperuricosuria....Pages 197-200
Renal Handling of Uric Acid in Gout by Means of the Pyrazinamide and Probenecid Tests....Pages 201-203
Behaviour of Serum Urate in Renal Disease of Varying Etiology....Pages 205-209
Hypouricemia and Medullary Carcinoma of the Thyroid....Pages 211-213
Purine Salvage Enzymes in Leishmania Donovani and Trichomonas Vaginalis ....Pages 215-218
Hypoxanthine Metabolism by Human Malaria Infected Erythrocytes: Focus for the Design of New Antimalarial Drugs....Pages 219-223
Adenosine Deaminase in Malaria Infection: Effect of 2′-Deoxycoformycin in Vivo ....Pages 225-229
Pyrazolopyrimidine Metabolism in Leishmania : An Overview....Pages 231-237
Single Cell Cloning of Leishmania Parasites in Purine-Defined Medium: Isolation of Drug-Resistant Variants....Pages 239-243
Selectivity of Antiviral Effectiveness Derived from Differences of Herpes Simplex Virus-Coded Thymidine Kinases....Pages 245-248
Adenosine Deaminase, Purine Nucleoside Phosphorylase and 5′-Nucleotidase Activities in Infectious Mononucleosis....Pages 249-252
T Cell Activation in Typhoid Fever Detected by Increased Levels of Adenosine Deaminase....Pages 253-256
Optimized Micro-Measurement of H.PRTase Activity on Fibroblasts and Hair Follicles....Pages 257-260
Optimal Conditions for Phosphoribosylpyrophosphate Synthesis in a Crude Extract of Liver Cells....Pages 261-264
Detection of Hypoxanthine Guanine Phosphoribosyl Transferase Heterozygotes by Thin Layer Chromatography and Autoradiography....Pages 265-268
A Rapid and Reliable Method for Separation of Ribonucleotides, Deoxynucleotides, and Cyclic Nucleotides by Reversed Phase HPLC....Pages 269-275
Turnover and Distribution of 14 C-Uric Acid in Psoriatic Patients....Pages 277-281
Serum Uric Acid Levels in Psoriasis....Pages 283-285
Purine Metabolites, Uracil and cAMP during Exchange Transfusion....Pages 287-290
Increases in Methylated Nucleosides during Human Pregnancy....Pages 291-295
Urinary Excretion of Methylated Nucleosides in Different States of Failure to Thrive....Pages 297-300
Regional Variation in Extracellular Purine Levels in Vivo....Pages 301-304
Lymphocyte Adenosine Deaminase Levels in Active and in Inactive Forms of HBsAg-Positive Chronic Liver Disease....Pages 305-308
Effect of Purine Restriction on Serum and Urine Urate in Normal Subjects....Pages 309-316
Isotope Studies of Uric Acid Metabolism During Dietary Purine Administration....Pages 317-321
Influence of Dietary Purines on Allopurinol Metabolism and Allopurinol Induced Oroticaciduria....Pages 323-326
The Effect of Beer Ingestion on Plasma and Urine Uric Acid in Gout and Normouricaemic Subjects....Pages 327-331
Influence of Purines on Pyrimidine Metabolism in Rat Hepatocytes....Pages 333-337
Effects of Glycerol on Purine Metabolism in Rat Liver Cells....Pages 339-342
Purine Release by Human Erythrocytes....Pages 343-346
Use of Adenine in Blood Banking....Pages 347-350
Substance Rates of Different Steps of Purine Metabolism in Normal and Preserved Red Blood Cells (RBC) Studied in Experiments Simulating In Vivo Conditions....Pages 351-354
Biochemical Study of a Case of Hemolytic Anemia with Increased (85-fold) Red Cell Adenosine Deaminase....Pages 355-358
Impaired Metabolism of Deoxyadenosine in Uremic Erythrocytes....Pages 359-362
Nucleotide Levels and Metabolism of Adenosine and Deoxyadenosine in Intact Erythrocytes Deficient in Adenosine Deaminase....Pages 363-366
Orotate Uptake and Metabolism by Human Erythrocytes....Pages 367-371
IMP Dehydrogenase Mutants: Cell Culture Model for Hyperuricemia....Pages 373-379
Basic Molecular Defect in ADA SCID....Pages 381-384
Genetic Mechanism(S) Responsible for a Deficiency of Adenine Phosphoribosyltransferase in Man....Pages 385-389
Correlation Between a Mutant APRT Protein and Altered DNA in CHO Cells....Pages 391-395
Purine, Thymidylate and Amino Acid Requirements for Human Lymphocyte Transformation and Fragile Chromosome Site Expression....Pages 397-400
Genetic Variability of Purine Nucleoside Phosphorylase in the Mouse....Pages 401-403
Deoxycoformycin Resistant Mammalian Cells That Overproduce Adenosine Deaminase....Pages 405-410
Characterization and Use of Cloned Sequences of the Hypoxanthine-Guanine Phosphoribosyltransferase Gene....Pages 411-415
Southern Analysis of the Lesch-Nyhan Locus in Man....Pages 417-420
De Novo Purine Synthesis in Human Lymphocytes....Pages 421-426
Human Phosphoribosylpyrophosphate (PRPP) Synthetase Requirements for Subunit Aggregation....Pages 427-432
The Hormonal Regulation of Purine Biosynthesis....Pages 433-436
The Hormonal Regulation of Purine Biosynthesis: Basal Levels of Different Hormones in Primary Gout....Pages 437-440
Regulation of De Novo Purine Biosynthesis in Chinese Hamster Cells....Pages 441-447
Regulatory Studies on Purine Biosynthesis in the Chick....Pages 449-452
The Chronologically Synchronous Elevation of Phosphoribosyl — Pyrophosphate and Cyclic AMP in Regenerating Rat Liver....Pages 453-456
Ethanol Induced Alterations of Uric Acid Metabolism....Pages 457-462
Influence of Ethanol on the Production of Allantoin by the Perfused Rat Liver....Pages 463-468
The Pathway of AMP Catabolism and Its Control in Isolated Rat Hepatocytes Subjected to Anoxia....Pages 469-474
Stimulation by Adenosine of Adenine Nucleotide Turnover in Isolated Hepatocytes: Evidence for a Futile Cycle Between AMP and Adenosine....Pages 475-480
Effects of Fructose on Purine Nucleotide Metabolism in Isolated Rat Hepatocytes....Pages 481-485
Effect of Hormones on Adenine and Adenosine Metabolism in Mammary Gland in Vitro....Pages 487-492
Nucleoside Utilization by S and G 1 Cells....Pages 493-496
Evidence for an Adenosine Receptor in Human Tissues....Pages 497-500
Adenosine Receptors on Human Inflammatory Cells....Pages 501-509
Biochemical Mechanisms for Sex Specific Differences of Rat Liver Xanthine Oxidase....Pages 511-517
Imprinting by the Neonatal Testis: A Mechanism for Sexual Dimorphism of Rat Liver Xanthine Oxidase....Pages 519-523
Pyrimidine Metabolism in Folate Deficient Lymphoblasts....Pages 525-529
Pyrimidine Metabolism in Rat Brain Cortex and Liver....Pages 531-534
Evidence for a Distinct Deoxypyrimidine 5′-Nucleotidase in Human Tissues....Pages 535-541
The Effect of 5-Fluoroorotic Acid on the Early Labelling of Nucleotides and RNA in Whole Liver and of RNA in Subnuclear Fractions in Rats Given 3 H-Orotic Acid....Pages 543-545
The Effect of Dipyridamole on Plasma Adenosine Levels and Skin Micro-Circulation in Man....Pages 547-547
Back Matter....Pages 549-555