Advances in Metabolic Disorders

Advances in Metabolic Disorders, Volume 1 presents the application of biochemistry to the pathogenesis of human diseases. This book discusses the parathormone physiology and its deviations, as well as the origins and course of osteoporosis. The introductory chapters deal with the catabolism of glycogen; physiology of parathyroids; multiple enzymic defects; biochemical technology of glycogen storage disease; assay of parathyroid hormone; mechanisms of mitochondrial respiratory control; pathogenesis of primary osteoporosis; and nature of bone change. The subsequent chapters consider the definition and physiology of insulin antagonists and chemistry of parathyroid hormone. These topics are followed by discussions of the physiologic effects of aldosterone and the metabolic interrelationship between folate and vitamin B12 are presented. A chapter is devoted to the definition and treatment of hypoparathyroidism and hyperparathyroidism. The remaining chapters focus on the diagnosis of insulin antagonists in tissues, plasma, and serum. The book can provide useful information to doctors, students, and researchers.

ContributorsPreface Glycogen Storage Disease I. Introduction II. The Catabolism of Glycogen III. General Considerations on Glycogen Storage Disease IV. Glycogenosis Related to a Phosphorylase Deficiency V. Limit Dextrinosis (Defect of Amylo-1, 6-Glucosidase) VI. Glycogenosis Related to the Absence of Glucose-6-Phosphatase VII. Glycogenosis Related to the Absence of Acid Maltase (Pompe's Disease) VIII. Amylopectinosis (Andersen's Disease) IX. Multiple Enzymic Defects X. Other Disturbances of Glycogen Metabolism XI. Biochemical Technology of Glycogen Storage Disease XII. General Discussion ReferencesThe Parathyroids I. Introduction II. Physiology III. Assay of Parathyroid Hormone IV. Chemistry of Parathyroid Hormone V. Biochemical Aspects of Parathyroid Hormone Action VI. Hyperparathyroidism VII. Hypoparathyroidism References Mitochondrial Respiratory Control: Biochemical PhysiologicalL, and Pathological Aspects Lars Ernster and Rolf Luft I. Introduction II. Biochemical Mechanisms III. Physiological and Pathological Aspects IV. Conclusions References Osteoporosis I. Definition and Classification II. Clinical Picture and Incidence III. Diagnosis IV. Nature of Bone Change V. Pathogenesis of Primary Osteoporosis VI. Secondary Osteoporosis VII. Therapy VIM. Conclusions References Basal Metabolic Rate and Thyroid Hormones I. Introduction II. The Determination of BMR III. Thyroid Hormones and the Regulation of BMR IV. Mode of Action of Thyroid Hormones References Insulin Antagonists and Inhibitors I. Introduction: Definition and Physiological Insulin Antagonists II. Insulin Antagonists in Tissues III. Insulin Antagonists in Plasma and Serum IV. Inhibitors of Glucose Uptake V. Discussion and Implications References Aldosterone: Its Biochemistry and Physiology I. Introduction II. Biochemistry III. Physiologic Effects of Aldosterone IV. Abnormal Aldosterone Secretion in Disease and Its Implications V. The Basic Problem: Mechanism for Normal Control of Aldosterone Secretion VI. Could Angiotensin Be the Trophic Hormone for Aldosterone? ReferencesFoloc Acid Deficiency in Man and its Interrelationship with VitaminN B12 Metabolism Part 1. Introduction Part 2. General and Historical Survey I. Historical Review of the Relationship of Folic Acid and Vitamin B12 to the Megaloblastic Anemias II. Distribution, Chemistry, and Absorption of Food Folate III. The Metabolic Functions of the Folate Coenzymes IV. The Interrelationships of the Folate Coenzymes V. Metabolic Reactions of Vitamin 812 and Site of Interaction with Folate in Mammals Part 3. Methodology I. Cytological Criteria of Megaloblastic Anemia II. The Therapeutic Trial As an Index of Specific Deficiency III. Newer Methods to Evaluate the Intermediary Metabolism and Physiology of Folates Part 4. Appraisal of Folate Deficiency and Metabolism in MegaloblasticAnemias and Other Clinical Disorders I. Clinical Diagnostic Experience with Various Parameters of Folate Deficiency II. Incidence of Folate Deficiency III. Nutritional Requirement and Metabolic Turnover of Folate IV. Metabolic Interrelationship between Folate and Vitamin References Addendum to the Paper "Glycogen Storage Disease"Author IndexSubject Index