The biochemistry of the pyruvate dehydrogenase complex

Biochemistry and Molecular Biology Education

Volumn 31, Issue 1, 2003, Pages 5-15

  Patel, Mulchand S ^a   Korotchkina, Lioubov G ^a  

^a UNIVERSITY AT BUFFALO   (United States)

Author keywords

Diabetes; Genetic defects; Pyruvate dehydrogenase complex; Regulation by phosphorylation

Indexed keywords

PYRUVATE DEHYDROGENASE;

AEROBIC METABOLISM; CITRIC ACID CYCLE; DIET; ENZYME STRUCTURE; GLUCOSE HOMEOSTASIS; GLYCOLYSIS; LACTATION; REVIEW;

EID: 4143097091     PISSN: 14708175     EISSN: None     Source Type: Journal    
DOI: 10.1002/bmb.2003.494031010156     Document Type: Review

References (45)

1. M. S. Patel, T. E. Roche, R. A. Harris, Eds. (1996) α-Keto Acid Dehydrogenase Complexes, Birkhauser Verlag, Basel, Switzerland.
2. M. C. Sugden, M. J. Holness (1994) Interactive regulation of the pyruvate dehydrogenase complex and the carnitine palmitoyltransferase system, FASEB J. 8, 54-61.
3. P. J. Randle, D. A. Priestman, S. C. Mistry, A. Halsall (1994) Glucose fatty acid interactions and the regulation of glucose disposal, J. Cell. Biochem. 55, 1-11.
4. M. S. Patel, R. A. Harris (1995) Mammalian α-keto acid dehydrogenase complexes: gene regulation and genetic defects, FASEB J. 9, 1164-1172.
5. R. N. Perham (2000) Swinging arms and swinging domains in multifunctional enzymes: catalytic machines for multistep reactions, Annu. Rev. Biochem. 69, 961-1004.
6. T. E. Roche, J. C. Baker, X. Yan, Y. Hiromasa, X. Gong, T. Peng, J. Dong, A. Turkan, S. A. Kasten (2001) Distinct regulatory properties of pyruvate dehydrogenase kinase and phosphatase isoforms, Prog. Nucleic Acid Res. Mol. Biol. 70, 33-75.
7. R. A. Harris, B. Huang, P. Wu (2001) Control of pyruvate dehydrogenase kinase gene expression, Adv. Enzyme Regul. 41, 269-288.
8. L. J. Reed (2001) A trail of research from lipoic acid to α-keto acid dehydrogenase complexes, J. Biol. Chem. 276, 38329-38336.
9. A. F. Hengeveld, A. deKok (2002) Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes, Curr. Med. Chem. 9, 499-520.
10. M. S. Patel, L. G. Korotchkina (2002) in Wiley Encyclopedia of Molecular Medicine, John Wiley and Sons, Inc., New York, Vol. 5, pp. 2699-2703.
11. R. A. Harris, M. M. Bowker-Kinley, B. Huang, P. Wu (2002) Regulation of the activity of the pyruvate dehydrogenase complex, Adv. Enzyme Regul. 42, 249-259.
12. M. S. Patel, T. E. Roche (1990) Molecular biology and biochemistry of pyruvate dehydrogenase complexes, FASEB J. 4, 3224-3233.
13. J. K. Stoops, R. H. Cheng, M. A. Yazdi, C. Y. Maeng, J. P. Schroeter, U. Klueppelberg, S. J. Kolodziej, T. S. Baker, L. J. Reed (1997) On the 3 unique structural organization of the Saccharomyces cerevisiae pyruvate dehydrogenase complex, J. Biol. Chem. 272, 5757-5764.
14. M. M. Klingbeil, D. J. Walker, R. Arnette, E. Sidawy, K. Hayton, P. R. Komuniecki, R. Komuniecki (1996) Identification of a novel dihydrolipoyl dehydrogenase-binding protein in the pyruvate dehydrogenase complex of the anaerobic parasitic nematode, Ascaris suum, J. Biol. Chem. 271, 5451-5457.
15. P. Arjunan, N. Nemeria, A. Brunskill, K. Chandrasekhar, M. Sax, Y. Yan, F. Jordan, J. R. Guest, W. Furey (2002) Structure of the pyruvate dehydrogenase multienzyme complex E1 component from Escherichia coli at 1.85 Å resolution, Biochemistry 41, 5213-5221.
16. S. S. Mande, S. Sarfaty, M. D. Allen, R. N. Perham, W. G. J. Hol (1996) Protein-protein interactions in the pyruvate dehydrogenase multienzyme complex: dihydrolipoamide dehydrogenase complexed with the binding domain of dihydrolipoamide acetyltransferase, Structure 4, 277-286.
17. Z. H. Zhou, W. Liao, R. H. Cheng, J. E. Lawson, D. B. McCarthy, L. J. Reed, J. K. Stoops (2001) Direct evidence for the size and conformational variability of the pyruvate dehydrogenase complex revealed by three-dimensional electron microscopy. The "breathing" core and its functional relationship to protein dynamics, J. Biol. Chem. 276, 21704-21713.
18. Z. H. Zhou, D. B. McCarthy, C. M. O'Connor, L. J. Reed, J. K. Stoops (2001) The remarkable structural and functional organization of the eukaryotic pyruvate dehydrogenase complexes, Proc. Natl. Acad. Sci. U. S. A. 98, 14802-14807.
19. A. Aevarsson, K. Seger, S. Turley, J. R. Sokatch, W. G. Hol (1999) Crystal structure of 2-oxoisovalerate and dehydrogenase and the architecture of 2-oxo acid dehydrogenase multienzyme complexes, Nat. Struct. Biol. 6, 785-792.
20. A. Aevarsson, J. L. Chuang, R. M. Wynn, S. Turley, D. T. Chuang, W. G. J. Hol (2000) Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease, Structure 8, 277-291.
21. A. Tripatara, L. G. Korotchkina, M. S. Patel (1999) Characterization of point mutations in patients with pyruvate dehydrogenase deficiency: role of methionine-181, proline-188, and arginine-349 in the α subunit, Arch. Biochem. Biophys. 367, 39-50.
22. S. J. Jacobia, L. G. Korotchkina, M. S. Patel (2001) Differential effects of two mutations at arginine-234 in the α subunit of human pyruvate dehydrogenase, Arch. Biochem. Biophys. 395, 121-128.
23. N. Nemeria, A. Volkov, A. Brown, J. Yi, L Zipper, J. R. Guest, F. Jordan (1998) Systematic study of the six cysteines of the E1 subunit of the pyruvate dehydrogenase multienzyme complex from Escherichia coli: none is essential for activity, Biochemistry 37, 911-922.
24. H.-H. M. Dahl, J. Fitzgerald, R. Iannelo, M. S. Patel, T. E. Roche, R. A. Harris, Eds. (1996) α-Keto Acid Dehydrogenase Complexes, Birkhauser Verlag, Basel, Switzerland, pp. 213-226.
25. Y. J. Huang, D. Walker, W. Chen, M. Klingbeil, R. Komuniecki (1998) Expression of pyruvate dehydrogenase isoforms during the aerobic/anaerobic transition in the development of the parasitic nematode Ascaris suum: altered stoichiometry of phosphorylation/inactivation, Arch. Biochem. Biophys. 352, 263-270.
26. L. G. Korotchkina, M. S. Patel (2001) Site specificity of four pyruvate dehydrogenase kinase isoenzymes toward the three phosphorylation sites of human pyruvate dehydrogenase, J. Biol. Chem. 276, 37223-37229.
27. M. S. Patel, L. G. Korotchkina (2001) Regulation of mammalian pyruvate dehydrogenase complex by phosphorylation: complexity of multiple phosphorylation sites and kinases, Exp. Mol. Med. 33, 191-197.
28. R. A. Harris, K. M. Popov, Y. Zhao, N. Y. Kedishvili, Y. Shimomura, D. W. Crabb (1995) A new family of protein kinases-the mitochondrial protein kinases, Adv. Enzyme Regul. 35, 147-162.
29. C. N. Steussy, K. M. Popov, M. M. Bowker-Kinley, R. B. Sloan, R. A. Harris, J. A. Hamilton (2001) Structure of pyruvate dehydrogenase kinase. Novel folding pattern for a serine protein kinase, J. Biol. Chem. 276, 37443-37450.
30. L. G. Korotchkina, M. S. Patel (2001) Probing the mechanism of inactivation of human pyruvate dehydrogenase by phosphorylation of three sites, J. Biol. Chem. 276, 5731-5738.
31. M. Caruso, M. A. Maitan, G. Bifulco, C. Miele, G. Vigliotta, F. Oriente, P. Formisano, F. Beguinot (2001) Activation and mitochondrial translocation of protein kinase Cδ are necessary for insulin stimulation of pyruvate dehydrogenase complex activity in muscle and liver cells, J. Biol. Chem. 276, 45088-45097.
32. M. H. Luethy, J. A. Miernyk, N. R. David, D. D. Randall, in M. S. Patel, T. E. Roche, R. A. Harris, Eds. (1996) α-Keto Acid Dehydrogenase Complexes, Birkhauser Verlag, Basel, Switzerland, pp. 71-92.
33. M. S. Patel, S. Naik, M. Johnson, R. Dey, in M. S. Patel, T. E. Roche, R. A. Harris, Eds. (1996) δ-Keto Acid Dehydrogenase Complexes, Birkhauser Verlag, Basel, Switzerland, pp. 197-211.
34. L. A. Da Silva, O. L. De Marcucci, Z. R. Kuhnle (1993) Dietary polyunsaturated fats suppress the high-sucrose-induced increase of rat liver pyruvate dehydrogenase levels, Biochim. Biophys. Acta 1169, 126-134.
35. J. Tan, H. S. Yang, M. S. Patel (1998) Regulation of mammalian pyruvate dehydrogenase δ subunit gene expression by glucose in HepG2 cells, Biochem. J. 336, 49-56.
36. M. C. Sugden, H. S. Lall, R. A. Harris, M. J. Holness (2000) Selective modification of the pyruvate dehydrogenase kinase isoform profile in skeletal muscle in hyperthyroidism: implications for the regulatory impact of glucose on fatty acid oxidation, J. Endocrinol. 167, 339-345.
37. M. J. Holness, N. D. Smith, K. Bulmer, T. Hopkins, G. F. Gibbons, M. C. Sugden (2002) Evaluation of the role of peroxisome-proliferator-activated receptor α in the regulation of cardiac pyruvate dehydrogenase kinase 4 protein expression in response to starvation, high-fat feeding and hyperthyroidism, Biochem. J. 364, 687-694.
38. B. Huang, K. M. Popov, R. A. Harris (2002) Expression of pyruvate dehydrogenase phosphatase 2 is decreased in diabetes and starvation, Diabetes 51, Suppl. 2, A136 (Abstr. 1288P).
39. M. Chang, S. Naik, G. L. Johanning, L. Ho, M. S. Patel (1993) Multiple protein-binding domains and functional cis-elements in the 5'′-flanking region of the human pyruvate dehydrogenase α-subunit gene, Biochemistry 32, 4263-4269.
40. R. Dey, S. Naik, M. S. Patel (1996) Tissue-specific expression of the human dehydrogenase a (Pdha-1)/chloramphenicol acetyltransferase fusion gene in transgenic mice, Biochim. Biophys. Acta 1305, 189-195.
41. R. C. Iannello, I. Kola, H. H. Dahl (1993) Temporal and tissue-specific interactions involving novel transcription factors and the proximal promoter of the mouse Pdha-2 gene, J. Biol. Chem. 268, 22581-22590.
42. U. Datta, I. D. Wexler, D. S. Kerr, I. Raz, M. S. Patel (1999) Characterization of the regulatory region of the human testis-specific form of the pyruvate dehydrogenase a-subunit (PDHA-2) gene, Biochim. Biophys. Acta 1447, 236-243.
43. D. S. Kerr, I. D. Wexler, A. Tripatara, M. S., Patel, in M. S. Patel, T. E. Roche, R. A. Harris, Eds. (1996) α-Keto Acid Dehydrogenase Complexes, Birkhauser, Basel, Switzerland, pp. 249-269.
44. W. Lissens, L. De Meirleir, S. Seneca, I. Liebaers, G. K. Brown, R. M. Brown, M. Ito, E. Naito, Y. Kuroda, D. S. Kerr, I. D. Wexler, M. S. Patel, B. H. Robinson, A. Seyda (2000) Mutations in the X-linked pyruvate dehydrogenase (E1) α subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency, Hum. Mutat. 15, 209-219.
45. M. T. Johnson, S. Mahmood, S. L. Hyatt, H. S. Yang, P. D. Soloway, R. W. Hanson, M. S. Patel (2001) Inactivation of the murine pyruvate dehydrogenase (Pdha1) gene and its effect on early embryonic development, Mol. Genet. Metab. 74, 293-302.