Thiamine: Catalytic Mechanisms in Normal and Disease States (Oxidative Stress and Disease)

Series Introduction......Page 6
Preface......Page 10
Contents......Page 12
Contributors......Page 18
Chemical Intermediates in Catalysis by Thiamine Diphosphate......Page 30
Mechanistic and Structural Studies on Thiamine Biosynthetic Enzymes......Page 44
Studies on the Structure and Function of Thiamine Pyrophosphokinase......Page 58
New Perspectives on the Cellular Role of Thiamine Triphosphate and Thiamine Triphosphatase......Page 72
How Thiamine Works in Enzymes: Time-Resolved NMR Snapshots of TDP-Dependent Enzymes in Action......Page 86
Thiamine-Dependent Enzymes as Catalysts of C–C Bond-Forming Reactions: The Role of ‘‘Orphan’’ Enzymes......Page 106
Ligand-Induced Conformational Changes in Thiamine Diphosphate–Dependent Enzymes: Comparison Between Crystal and Solution Structures......Page 122
Enantioselective Syntheses of Hydroxy Ketones via Benzoylformate Decarboxylase- and Benzaldehyde Lyase-Catalyzed C–C Bond Formation......Page 142
Benzoylformate Decarboxylase: Lessons in Enzymology......Page 160
New Concept on the Nature of the Induced Absorption Band of Holotransketolase......Page 172
Structure of the α-Carbanion/Enamine Reaction Intermediate in the Active Site of Transketolase, Determined by Kinetic Crystallography......Page 188
Yeast Pyruvate Decarboxylase: New Features of the Structure and Mechanism......Page 202
Solvent and Carbon Kinetic Isotope Effects on Active-Site and Regulatory-Site Variants of Yeast Pyruvate Decarboxylase......Page 246
Insights into the Mechanism and Regulation of Bacterial Acetohydroxyacid Synthases......Page 262
Structure and Properties of Acetohydroxyacid Synthase......Page 280
Exploring the Substrate Specificity of Benzoylformate Decarboxylase, Pyruvate Decarboxylase, and Benzaldehyde Lyase......Page 304
Benzoylformate Decarboxylase: Intermediates, Transition States, and Diversions......Page 320
Structural and Functional Organization of Pyruvate Dehydrogenase Complexes......Page 338
The Pyruvate Dehydrogenase Multienzyme Complex......Page 360
Activation and Transfer of Lipoic Acid in Protein Lipoylation in Mammals......Page 372
Central Organization of Mammalian Pyruvate Dehydrogenase (PD) Complex and Lipoyl Domain–Mediated Activated Function and Control of PD Kinases and Phosphatase 1......Page 403
Physiological Effects of Replacing the PDH Complex of E. coli by Genetically Engineered Variants or by Pyruvate Oxidase......Page 427
Structure and Intersubunit Information Transfer in the E. coli Pyruvate Dehydrogenase Multienzyme Complex......Page 447
Structure, Function, and Regulation of Pyruvate Dehydrogenase Kinase......Page 473
Three-Dimensional Structures for Components and Domain of the Mammalian Branched-Chain α-Ketoacid Dehydrogenase Complex......Page 493
Variability of Human Pyruvate Dehydrogenase Complex Deficiency......Page 515
Kinetic Studies of Human Pyruvate Dehydrogenase and Its Mutants: Interactions with Thiamine Pyrophosphate......Page 529
The Complexity of Single-Gene Disorders: Lessons from Maple Syrup Urine Disease and Thiamine Responsiveness......Page 553
Thiamine Pyrophosphate: An Essential Cofactor in the Mammalian Metabolism of 3-Methyl-Branched Fatty Acids......Page 569
Pathogenesis of Selective Neuronal Loss in Wernicke–Korsakoff Syndrome: Role of Oxidative Stress......Page 583
Thiamine-Responsive Megaloblastic Anemia Syndrome: Clinical Aspects and Molecular Genetics......Page 593
Accomplishments and Future Directions......Page 609
Index......Page 613