Alpha-Keto Acid Dehydrogenase Complexes

  • Mulchand S. Patel
  • Thomas E. Roche
  • Robert A. Harris

Part of the MCBU Molecular and Cell Biology Updates book series (MCBU)

Table of contents

  1. Front Matter
    Pages I-IX
  2. L. G. Korotchkina, M. S. Ali, M. S. Patel
    Pages 17-32
  3. A. de Kok, W. J. H. van Berkel
    Pages 53-70
  4. M. H. Luethy, J. A. Miernyk, N. R. David, D. D. Randall
    Pages 71-92
  5. R. M. Wynn, J. R. Davie, M. Meng, D. T. Chuang
    Pages 101-117
  6. Y. Motokawa, K. Fujiwara, K. Okamura-Ikeda
    Pages 119-130
  7. L. J. Reed, J. E. Lawson, X.-D. Niu, J. Yan
    Pages 131-138
  8. M. C. Sugden, M. J. Holness
    Pages 163-176
  9. H.-H. M. Dahl, J. Fitzgerald, R. Iannello
    Pages 213-226
  10. D. S. Kerr, I. D. Wexler, A. Tripatara, M. S. Patel
    Pages 249-269
  11. Back Matter
    Pages 319-321

About this book


Found in all organisms, the alpha-keto acid dehydrogenase complexes have central roles in cellular metabolism and are major sites of regulation. The understanding of the organization, function and regulation of these quintessential multienzyme complexes has been greatly advanced by studies employing molecular biology and biophysical techniques. Although these enzyme systems have some features in common, their diversity in fulfilling unique organism - or tissue - specific roles is truly amazing. These systems have medical importance in areas ranging from defects in regulation (linked to diabetes, heart disease, obesity, nutrition defects), to inherited diseases (inborn errors, maple syrup urine disease) to acquired immune diseases (primary biliary cirrhosis). This book brings together wide-ranging recent findings on the structure(function relationships, gene regulation, and genetic defects of the alpha-keto acid dehydrogenase complexes, namely the pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase and the branched-chain alpha-keto acid dehydrogenase complexes. A wide variety of experimental approaches together with new results presented in this book should serve as a resource for beginning to established investigators in the field as well as scientists who are interested in mitochondria, dehydrogenases, kinases, phosphatases, lipoic acid, thiamine pyrophosphate, and enzyme complexes.


Molekularbiologie biochemistry biology enzyme enzymes gene medicine metabolism mitochondria molecular biology mutation primary structure protein regulation tissue

Editors and affiliations

  • Mulchand S. Patel
    • 1
  • Thomas E. Roche
    • 2
  • Robert A. Harris
    • 3
  1. 1.Department of Biochemistry School of Medicine and Biomedical SciencesState University of New YorkBuffaloUSA
  2. 2.Department of BiochemistryKansas State UniversityManhattanUSA
  3. 3.Department of Biochemistry and Molecular BiologyIndiana University School of MedicineIndianapolisUSA

Bibliographic information