Vitamin-Dependent Multienzyme Complexes of 2-Oxo Acid Dehydrogenases: Structure, Function, Regulation and Medical Implications

Victoria Bunik
Principal Investigator of Belozersky Institute of Physicochemical Biology and Associate Professor of Faculty of Bioengineering and Bioinformatics, Lomonosov Moscow State University, Moscow, Russian Federation

Series: Biochemistry Research Trends
BISAC: SCI007000

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This book summarizes current data on the architecture, structure-function relationships, reaction mechanisms and active site coupling in multienzyme complexes of 2-oxo acid dehydrogenases. Special focus concerns the mechanisms of function and regulation of a multienzyme structure, additional to those inherent in its component enzymes. The overall physiological process of 2-oxo acid oxidative decarboxylation and the substrate specificity determinants are considered along with side reactions of regulatory significance, such as non-oxidative reactions, formation of reactive oxygen species (ROS) and complex-bound thiyl radicals. The significance of the chemical interplay between the complex-bound dihydrolipoate intermediate, thioredoxin or thioredoxin-like proteins, and peroxiredoxins for sensing metabolic state and adjusting the catalysis accordingly, is considered. Other mechanisms involved in the short- and long-term regulation of 2-oxo acid dehydrogenase complexes are summarized in view of their relevance to physiological functions. Metabolic regulation by natural effectors of 2-oxo acid dehydrogenase complexes and their synthetic analogues is considered. The medical implications of enzymological knowledge on the complexes are also presented. (Imprint: Nova)

Preface

Abbreviations

Chapter 1. Introduction

Chapter 2. Principles of Self-Assembly of 2-Oxo Acid Dehydrogenase Complexes

Chapter 3. Catalytic Mechanism of Physiological 2-Oxo Acid Dehydrogenase Reaction

Chapter 4. Side Reactions Catalysed By 2-Oxo Acid Dehydrogenase Complexes

Chapter 5. Functional Significance of Variable Component Stoichiometry of 2-Oxo Acid Dehydrogenase Complexes

Chapter 6. Principles of Regulation of 2-Oxo Acid Dehydrogenase complexes

Chapter 7. Medical Implications of 2-Oxo Acid Dehydrogenase Complexes

Conclusion

Acknowledgments

References

About the Author

Index

“Dr. Bunik has produced a comprehensive, detailed and authoritative account of the biochemistry, enzymology and regulation of this important family of multienzyme assemblies that control key steps in the major pathways of central metabolism. Their involvement in oxidative stress responses and disease states including cancer and neuropathological conditions linked to glutamate toxicity and acetylcholine synthesis is also discussed in detail. This book represents a distinctive and significant contribution to this important area of research, providing an ideal source of information for new or experienced researchers alike.” - John Gordon Lindsay, Ph.D., Professor of Medical Biochemistry (Honorary), University of Glasgow, Institute of Molecular, Cell and Systems Biology, Glasgow, Scotland, UK

"As a life-long enzyme enthusiast and scholar of thiamine and redox biology, I am captivated by the novel insights that Victoria Bunik has narrated in her monograph on vitamin-dependent multienzyme complexes. This work presents new dimensions in the actions of the age-old B-vitamin, thiamine, and delineates the interdependent actions of the various catalytic functions of the 2-oxo acid dehydrogenases, characterizing rate-determining conformational changes in the macromolecular structure and distinguishing key progressive reactions from side reactions. Difficult mechanistic details of catalysis such as the role of thiyl radical intermediates are portrayed with skill. Besides regulation of gene expression, an in-depth consideration of various mechanisms of post-translational regulation of the enzyme complex provides a valuable resource for investigators entering this field of study. Finally, the functional importance of these enzyme complexes in health and disease is brought to the fore. This monograph by Bunik is a scholarly tour de force which will capture the interest and satisfy the creative imagination of investigators and students over a broad range of the biomedical sciences." - John J. Mieyal, Ph.D., Professor and Vice-Chairman of Pharmacology, Case Western Reserve University, Cleveland, Ohio USA

"Victoria Bunik, who worked successfully also here in Tuebingen, wrote this excellent book, comprehensive and always up to date in any respect. Never I read a better book in this important field. It is absolute worthwhile to read it!" - Peter Bohley, Professor of Biochemistry, Emeritus, Interfakultaeres Institut fuer Biochemie, Tuebingen, Germany

"This book is an important contribution by the world leader in this area. Interest in the 2-oxo acid dehydrogenases has exploded. New and powerful tools for studying their structure and function have stimulated new understanding. Roles for these enzymes beyond energy metabolism and ROS production including regulation of protein cross talk and transcription are now apparent. In addition, we now know these enzymes have a surprisingly key role in cancer, stem cell biology, neurodegeneration and many other areas. In combination, these new ideas have created a large literature that requires the kinds of syntheses that has been accomplished in this outstanding book." - Gary E. Gibson, Ph.D., Professor, Brain and Mind Research Institute Weill Cornell Medicine, Burke Medical Research Institute, NY, USA

"In her concise book prof. Victoria Bunik summarizes comprehensive information on multienzyme complexes of the dehydrogenases of pyruvate, 2-oxoglutarate and branched-chain 2-oxo acids. Unique nature of the molecular conveyers, with their usage of multiple vitamin derivatives and precise regulation, is shown to be appropriate for metabolic checkpoints which the complexes occupy." - Slawomir Strumilo, PhD, Dr.Sci., Professor of Biochemistry, University of Bialystok, Poland

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