Thiamine: Catalytic Mechanisms in Normal and Disease States brings together the most recent developments in thiamine diphosphate (TDP)-requiring enzyme research and details the mechanisms of catalysis and structure-function relationships, as well as pathophysiological aspects of a spectrum of diseases associated with TDP-requiring enzymes. Providin

Series Introduction, Preface, Contributors, Part I: Introduction, 1. Chemical Intermediates in Catalysis by Thiamine Diphosphate, Part II: Biosynthesis of Thiamine and Its Phosphorylated Terms 2. Mechanistic and Structural Studies on Thiamine Biosynthetic Enzymes, 3. Studies on the Structure and Function of Thiamine Pyrophosphokinase, 4. New Perspectives on the Cellular Role of Thiamine Triphosphate and Thiamine Triphosphatase, Part III: Enzymology of Thiamine Diphosphate Enzymes, 5. How Thiamine Works in Enzymes: Time-Resolved NMR Snapshots of TDP-Dependent Enzymes in Action, 6. Thiamine-Dependent Enzymes as Catalysts of C C Bond-Forming Reactions: The Role of "Orphan" Enzymes, 7. Ligand-Induced Conformational Changes in Thiamine Diphosphate-Dependent Enzymes: Comparison Between Crystal and Solution Structures, 8. Enantioselective Synthesis of Hydroxy Ketones via Benzoylformate Decarboxylase and Benzaldehyde Lyase-Catalyzed C-C Bond Formation, 9. Benzoylformate Decarboxylase: Lessons in Enzymology, 10. New Concept on the Nature of the Induced Absorption Band of Holotransketolase, 11. Structure of the a-Carbanion/Enamine Reaction Intermediate in the Active Site of Transketolase, Determined by Kinetic Crystallography, 12. Yeast Pyruvate Decarboxylase: New Features of the Structure and Mechanism, 13. Solvent and Carbon Kinetic Isotope Effects on Active-Site and Regulatory Site Variants of Yeast Pyruvate Decarboxylase, 14. Insights into the Mechanism and Regulation of Bacterial Acetohydroxyacid Synthases, 15. Structure and Properties of Acetohydroxyacid Synthase, 16. Exploring the Substrate Specificity of Benzoylformate Decarboxylase, Pyruvate Decarboxylase, and Benzaldehyde Lyase, 17. Benzoylformate Decarboxylase: Intermediates, Transition States, and Diversions, Part IV: Structure and Function of Thiamine Diphosphate Multienzyme Complexes, 18. Structural and Functional Organization of PyruvateDehydrogenase Complexes, 19. The Pyruvate Dehydrogenase Multienzyme Complex, 20. Activation and Transfer of Lipoic Acid in Protein Lipoylation in Mammals, 21. Central Organization of Mammalian Pyruvate Dehydrogenase (PD) Complex and Lipoyl Domain-Mediated Activated Function and Control of PD Kinases and Phosphatase, 22. Physiological Effects of Replacing the PDH Complex of E. coli by Genetically Engineered Variants or by Pyruvate Oxidase, 23. Structure and Intersubunit Information Transfer in the E. coli Pyruvate Dehydrogenase Multienzyme Complex, 24. Structure, Function, and Regulation of Pyruvate Dehydrogenase Kinase, 25. Three-Dimensional Structures for Components and Domain of the Mammalian Branched-Chain a-Ketoacid Dehydrogenase Complex, Part V: Biomedical Aspects of Thiamine Diphosphate Dependent Enzymes, 26. Variability of Human Pyruvate Dehydrogenase Complex Deficiency, 27. Kinetic Studies of Human Pyruvate Dehydrogenase and Its Mutants: Interactions with Thiamine Pyrophosphate, 28. The Complexity of Single-Gene Disorders: Lessons from Maple Syrup Urine Disease and Thiamine Responsiveness, 29. Thiamine Pyrophosphate: An Essential Cofactor in the Mammalian Metabolism of 3-Methyl-Branched Fatty Acids, 30. Pathogenesis of Selective Neuronal Loss in Wemicke Korsakoff Syndrome: Role of Oxidative Stress, 31. Thiamine-Responsive Megaloblastic Anemia Syndrome: Clinical Aspects and Molecular Genetics, Part VI: Concluding Remarks, 32. Accomplishments and Future Directions, Index

Frank Jordan is Rutgers University Board of Governors Professor of Chemistry, Rutgers University, Newark, New Jersey. The author or coauthor of more than 185 professional publications and the holder of three patents, Dr. Jordan is a member of the American Society for Biochemistry and Molecular Biology, the American Chemical Society, and the American Association for the Advancement of Science. Dr. Jordan received the B.S. degree (1964) in chemistry from Drexel University, Philadelphia, Pennsylvania; the Ph.D. degree (1967) in chemistry from the University of Pennsylvania, Philadelphia; a NATO Postdoctoral Fellowship (1968), University of Paris, France; and an NIH Postdoctoral Fellowship (1970), Harvard University, Cambridge, Massachusetts.

Mulchand S. Patel is Professor of Biochemistry and Associate Dean for Biomedical Research and Education, School of Medicine and Biomedical Sciences, State University of New York at Buffalo, New York. The coeditor of three books and author of more than 180 professional publications, Dr. Patel is a member of the American Society for Biochemistry and Molecular Biology, the American Society for Nutritional Sciences, and the American Association for the Advancement of Science. Dr. Patel received the B.Sc. degree (1961) in biochemistry from M.S. University, Baroda, India, and the Ph.D. degree (1968) in animal science from the University of Illinois at Urbana-Champaign.