• 2510 Citations
  • 31 h-Index
1982 …2019

Research output per year

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Research Output

  • 2510 Citations
  • 31 h-Index
  • 88 Article
  • 1 Chapter
2019

In vivo assessment of increased oxidation of branched-chain amino acids in glioblastoma

Suh, E. H., Hackett, E. P., Wynn, R. M., Chuang, D. T., Zhang, B., Luo, W., Sherry, A. D. & Park, J. M., Dec 1 2019, In : Scientific Reports. 9, 1, 340.

Research output: Contribution to journalArticle

1 Scopus citations
Open Access

Targeting BCAA catabolism to treat obesity-associated insulin resistance

Zhou, M., Shao, J., Wu, C. Y., Shu, L., Dong, W., Liu, Y., Chen, M., Wynn, R. M., Wang, J., Wang, J., Gui, W. J., Qi, X., Lusis, A. J., Li, Z., Wang, W., Ning, G., Yang, X., Chuang, D. T., Wang, Y. & Sun, H., Sep 1 2019, In : Diabetes. 68, 9, p. 1730-1746 17 p.

Research output: Contribution to journalArticle

Open Access
7 Scopus citations

Therapeutic Effect of Targeting Branched-Chain Amino Acid Catabolic Flux in Pressure-Overload Induced Heart Failure

Chen, M., Gao, C., Yu, J., Ren, S., Wang, M., Wynn, R. M., Chuang, D. T., Wang, Y. & Sun, H., Jun 4 2019, In : Journal of the American Heart Association. 8, 11, e011625.

Research output: Contribution to journalArticle

Open Access
2018

A novel inhibitor of pyruvate dehydrogenase kinase stimulates myocardial carbohydrate oxidation in diet-induced obesity

Wu, C. Y., Satapati, S., Gui, W., Max Wynn, R., Sharma, G., Lou, M., Qi, X., Burgess, S. C., Malloy, C., Khemtong, C., Dean Sherry, A., Chuang, D. T. & Merritt, M. E., Jan 1 2018, In : Journal of Biological Chemistry. 293, 25, p. 9604-9613 10 p.

Research output: Contribution to journalArticle

3 Scopus citations

Targeting hepatic pyruvate dehydrogenase kinases restores insulin signaling and mitigates ChREBP-mediated lipogenesis in diet-induced obese mice

Wu, C. Y., Tso, S. C., Chuang, J. L., Gui, W. J., Lou, M., Sharma, G., Khemtong, C., Qi, X., Wynn, R. M. & Chuang, D. T., Jan 1 2018, (Accepted/In press) In : Molecular Metabolism.

Research output: Contribution to journalArticle

9 Scopus citations

The BCKDH Kinase and Phosphatase Integrate BCAA and Lipid Metabolism via Regulation of ATP-Citrate Lyase

White, P. J., McGarrah, R. W., Grimsrud, P. A., Tso, S. C., Yang, W. H., Haldeman, J. M., Grenier-Larouche, T., An, J., Lapworth, A. L., Astapova, I., Hannou, S. A., George, T., Arlotto, M., Olson, L. B., Lai, M., Zhang, G. F., Ilkayeva, O., Herman, M. A., Wynn, R. M., Chuang, D. T. & 1 others, Newgard, C. B., Jan 1 2018, (Accepted/In press) In : Cell Metabolism.

Research output: Contribution to journalArticle

36 Scopus citations
2017

Development of Dihydroxyphenyl Sulfonylisoindoline Derivatives as Liver-Targeting Pyruvate Dehydrogenase Kinase Inhibitors

Tso, S. C., Lou, M., Wu, C. Y., Gui, W. J., Chuang, J. L., Morlock, L. K., Williams, N. S., Wynn, R. M., Qi, X. & Chuang, D. T., Feb 9 2017, In : Journal of Medicinal Chemistry. 60, 3, p. 1142-1150 9 p.

Research output: Contribution to journalArticle

12 Scopus citations

Identification of the ferredoxin interaction sites on ferredoxin-dependent glutamate synthase from Synechocystis sp. PCC 6803

Hirasawa, M., Solis, J., Vaidyanathan, N., Srivastava, A. P., Wynn, R. M., Sutton, R. B. & Knaff, D. B., Oct 3 2017, (Accepted/In press) In : Photosynthesis Research. p. 1-12 12 p.

Research output: Contribution to journalArticle

1 Scopus citations
2016

Catabolic defect of branched-chain amino acids promotes heart failure

Sun, H., Olson, K. C., Gao, C., Prosdocimo, D. A., Zhou, M., Wang, Z., Jeyaraj, D., Youn, J. Y., Ren, S., Liu, Y., Rau, C. D., Shah, S., Ilkayeva, O., Gui, W. J., William, N. S., Wynn, R. M., Newgard, C. B., Cai, H., Xiao, X., Chuang, D. T. & 4 others, Schulze, P. C., Lynch, C., Jain, M. K. & Wang, Y., May 24 2016, In : Circulation. 133, 21, p. 2038-2049 12 p.

Research output: Contribution to journalArticle

107 Scopus citations

Metabolite regulation of nuclear localization of carbohydrate-response element-binding protein (ChREBP): Role of amp as an allosteric inhibitor

Sato, S., Jung, H., Nakagawa, T., Pawlosky, R., Takeshima, T., Lee, W. R., Sakiyama, H., Laxman, S., Wynn, R. M., Tu, B. P., MacMillan, J. B., De Brabander, J. K., Veech, R. L. & Uyeda, K., May 13 2016, In : Journal of Biological Chemistry. 291, 20, p. 10515-10527 13 p.

Research output: Contribution to journalArticle

22 Scopus citations
2015

GM130 Regulates Golgi-Derived Spindle Assembly by Activating TPX2 and Capturing Microtubules

Wei, J. H., Zhang, Z. C., Wynn, R. M. & Seemann, J., Jul 18 2015, In : Cell. 162, 2, p. 287-299 13 p., 8260.

Research output: Contribution to journalArticle

46 Scopus citations
2014

Benzothiophene carboxylate derivatives as novel allosteric inhibitors of branched-chain α-ketoacid dehydrogenase kinase

Tso, S. C., Gui, W. J., Wu, C. Y., Chuang, J. L., Qi, X., Skvorak, K. J., Dorko, K., Wallace, A. L., Morlock, L. K., Lee, B. H., Hutson, S. M., Strom, S. C., Williams, N. S., Tambar, U. K., Wynn, R. M. & Chuang, D. T., Jul 25 2014, In : Journal of Biological Chemistry. 289, 30, p. 20583-20593 11 p.

Research output: Contribution to journalArticle

19 Scopus citations

Maple Syrup Urine Disease: Clinical and Therapeutic Considerations

Chuang, D. T., Max Wynn, R., Cox, R. P. & Chuang, J. L., Nov 13 2014, Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease: Fifth Edition. Elsevier Inc., p. 663-672 10 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Structure-guided development of specific pyruvate dehydrogenase kinase inhibitors targeting the ATP-binding pocket

Tso, S. C., Qi, X., Gui, W. J., Wu, C. Y., Chuang, J. L., Wernstedt-Asterholm, I., Morlock, L. K., Owens, K. R., Scherer, P. E., Williams, N. S., Tambar, U. K., Wynn, R. M. & Chuang, D. T., Feb 14 2014, In : Journal of Biological Chemistry. 289, 7, p. 4432-4443 12 p.

Research output: Contribution to journalArticle

48 Scopus citations
2013

Metabolite regulation of nucleo-cytosolic trafficking of carbohydrate response element-binding protein (ChREBP): Role of ketone bodies

Nakagawa, T., Ge, Q., Pawlosky, R., Wynn, R. M., Veech, R. L. & Uyeda, K., Sep 27 2013, In : Journal of Biological Chemistry. 288, 39, p. 28358-28367 10 p.

Research output: Contribution to journalArticle

23 Scopus citations

Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain α-ketoacid dehydrogenase kinase

Tso, S. C., Qi, X., Gui, W. J., Chuang, J. L., Morlock, L. K., Wallace, A. L., Ahmed, K., Laxman, S., Campeau, P. M., Lee, B. H., Hutson, S. M., Tu, B. P., Williams, N. S., Tambar, U. K., Wynn, R. M. & Chuang, D. T., Jun 11 2013, In : Proceedings of the National Academy of Sciences of the United States of America. 110, 24, p. 9728-9733 6 p.

Research output: Contribution to journalArticle

28 Scopus citations
2012

Structural and biochemical characterization of human mitochondrial branched-chain α-ketoacid dehydrogenase phosphatase

Wynn, R. M., Li, J., Brautigam, C. A., Chuang, J. L. & Chuang, D. T., Mar 16 2012, In : Journal of Biological Chemistry. 287, 12, p. 9178-9192 15 p.

Research output: Contribution to journalArticle

15 Scopus citations

Structural characterization of a unique interface between carbohydrate response element-binding protein (ChREBP) and 14-3-3β protein

Ge, Q., Huang, N., Wynn, R. M., Li, Y., Du, X., Miller, B., Zhang, H. & Uyeda, K., Dec 7 2012, In : Journal of Biological Chemistry. 287, 50, p. 41914-41921 8 p.

Research output: Contribution to journalArticle

16 Scopus citations
2011

Importin-α protein binding to a nuclear localization signal of carbohydrate response element-binding protein (ChREBP)

Ge, Q., Nakagawa, T., Max Wynn, R., Chook, Y. M., Miller, B. C. & Uyeda, K., Aug 12 2011, In : Journal of Biological Chemistry. 286, 32, p. 28119-28127 9 p.

Research output: Contribution to journalArticle

21 Scopus citations

Phenylbutyrate therapy for maple syrup urine disease

Brunetti-pierri, N., Lanpher, B., Erez, A., Ananieva, E. A., Islam, M., Marini, J. C., Sun, Q., Yu, C., Hegde, M., Li, J., Wynn, R. M., Chuang, D. T., Hutson, S. & Lee, B., Feb 2011, In : Human Molecular Genetics. 20, 4, p. 631-640 10 p., ddq507.

Research output: Contribution to journalArticle

42 Scopus citations
15 Scopus citations
2010

Branched-chain amino acid metabolon: Interaction of glutamate dehydrogenase with the mitochondrial branched-chain aminotransferase (BCATm)

Islam, M. M., Nautiyal, M., Wynn, R. M., Mobley, J. A., Chuang, D. T. & Hutson, S. M., Jan 1 2010, In : Journal of Biological Chemistry. 285, 1, p. 265-276 12 p.

Research output: Contribution to journalArticle

55 Scopus citations
22 Scopus citations

Determination of protein complex stoichiometry through multisignal sedimentation velocity experiments

Padrick, S. B., Deka, R. K., Chuang, J. L., Wynn, R. M., Chuang, D. T., Norgard, M. V., Rosen, M. K. & Brautigam, C. A., Dec 2010, In : Analytical Biochemistry. 407, 1, p. 89-103 15 p.

Research output: Contribution to journalArticle

31 Scopus citations
2009

Maple syrup urine disease in Cypriot families: identification of three novel mutations and biochemical characterization of the p.Thr211Met mutation in the E1alpha subunit.

Georgiou, T., Chuang, J. L., Wynn, R. M., Stylianidou, G., Korson, M., Chuang, D. T. & Drousiotou, A., Oct 2009, In : Genetic Testing and Molecular Biomarkers. 13, 5, p. 657-664 8 p.

Research output: Contribution to journalArticle

4 Scopus citations
32 Scopus citations
2008

Pyruvate dehydrogenase kinase-4 structures reveal a metastable open conformation fostering robust core-free basal activity

Wynn, R. M., Kato, M., Chuang, J. L., Tso, S. C., Li, J. & Chuang, D. T., Sep 12 2008, In : Journal of Biological Chemistry. 283, 37, p. 25305-25315 11 p.

Research output: Contribution to journalArticle

46 Scopus citations

Regulation of nuclear import/export of carbohydrate response element-binding protein (ChREBP): Interaction of anα-helix of ChREBP with the 14-3-3 proteins and regulation by phosphorylation

Sakiyama, H., Wynn, R. M., Lee, W. R., Fukasawa, M., Mizuguchi, H., Gardner, K. H., Repa, J. J. & Uyeda, K., Sep 5 2008, In : Journal of Biological Chemistry. 283, 36, p. 24899-24908 10 p.

Research output: Contribution to journalArticle

60 Scopus citations

Structural Basis for Inactivation of the Human Pyruvate Dehydrogenase Complex by Phosphorylation: Role of Disordered Phosphorylation Loops

Kato, M., Wynn, R. M., Chuang, J. L., Tso, S. C., Machius, M., Li, J. & Chuang, D. T., Dec 12 2008, In : Structure. 16, 12, p. 1849-1859 11 p.

Research output: Contribution to journalArticle

53 Scopus citations
2007

A novel branched-chain amino acid metabolon: Protein-protein interactions in a supramolecular complex

Islam, M. M., Wallin, R., Wynn, R. M., Conway, M., Fujii, H., Mobley, J. A., Chuang, D. T. & Hutson, S. M., Apr 20 2007, In : Journal of Biological Chemistry. 282, 16, p. 11893-11903 11 p.

Research output: Contribution to journalArticle

70 Scopus citations

The two active sites in human branched-chain α-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanism

Li, J., Machius, M., Chuang, J. L., Wynn, R. M. & Chuang, D. T., Apr 20 2007, In : Journal of Biological Chemistry. 282, 16, p. 11904-11913 10 p.

Research output: Contribution to journalArticle

10 Scopus citations
2006

A synchronized substrate-gating mechanism revealed by cubic-core structure of the bovine branched-chain α-ketoacid dehydrogenase complex

Kato, M., Wynn, R. M., Chuang, J. L., Brautigam, C. A., Custorio, M. & Chuang, D. T., Dec 13 2006, In : EMBO Journal. 25, 24, p. 5983-5994 12 p.

Research output: Contribution to journalArticle

21 Scopus citations

A versatile conformational switch regulates reactivity in human branched-chain α-ketoacid dehydrogenase

Machius, M., Wynn, R. M., Chuang, J. L., Li, J., Kluger, R., Yu, D., Tomchick, D. R., Brautigam, C. A. & Chuang, D. T., Feb 2006, In : Structure. 14, 2, p. 287-298 12 p.

Research output: Contribution to journalArticle

40 Scopus citations

Erratum: A versatile conformational switch regulates reactivity in human branched-chain α-ketoacid dehydrogenase (Structure DOI:10.1016/j.str.2006. 03.001)

Machius, M., Wynn, R. M., Chuang, J. L., Li, J., Kluger, R., Yu, D., Tomchick, D. R., Brautigam, C. A. & Chuang, D. T., Mar 2006, In : Structure. 14, 3, p. 625 1 p.

Research output: Contribution to journalArticle

Lessons from genetic disorders of branched-chain amino acid metabolism

Chuang, D. T., Chuang, J. L. & Wynn, R. M., Jan 2006, In : Journal of Nutrition. 136, 1

Research output: Contribution to journalArticle

75 Scopus citations
58 Scopus citations
2005

Crystal structure of pyruvate dehydrogenase kinase 3 bound to lipoyl domain 2 of human pyruvate dehydrogenase complex

Kato, M., Chuang, J. L., Tso, S. C., Wynn, R. M. & Chuang, D. T., May 18 2005, In : EMBO Journal. 24, 10, p. 1763-1774 12 p.

Research output: Contribution to journalArticle

71 Scopus citations
2004

Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain α-keto acid decarboxylase/ dehydrogenase

Li, J., Wynn, R. M., Machius, M., Chuang, J. L., Karthikeyan, S., Tomchick, D. R. & Chuang, D. T., Jul 30 2004, In : Journal of Biological Chemistry. 279, 31, p. 32968-32978 11 p.

Research output: Contribution to journalArticle

28 Scopus citations

Molecular mechanism for regulation of the human mitochondrial branched-chain α-ketoacid dehydrogenase complex by phosphorylation

Wynn, R. M., Kato, M., MacHius, M., Chuang, J. L., Li, J., Tomchick, D. R. & Chuang, D. T., Dec 2004, In : Structure. 12, 12, p. 2185-2196 12 p.

Research output: Contribution to journalArticle

44 Scopus citations

Structural and biochemical basis for novel mutations in homozygous Israeli maple syrup urine disease patients: A proposed mechanism for the thiamin-responsive phenotype

Chuang, J. L., Wynn, R. M., Moss, C. C., Song, J. L., Li, J., Awad, N., Mandel, H. & Chuang, D. T., Apr 23 2004, In : Journal of Biological Chemistry. 279, 17, p. 17792-17800 9 p.

Research output: Contribution to journalArticle

34 Scopus citations
2003
14 Scopus citations
2002
14 Scopus citations
2001

Biochemical basis of type IB (E1β) mutations in maple syrup urine disease. A prevalent allele in patients from the druze kindred in Israel

Wynn, R. M., Chuang, J. L., Sansaricq, C., Mandel, H. & Chuang, D. T., Sep 28 2001, In : Journal of Biological Chemistry. 276, 39, p. 36550-36556 7 p.

Research output: Contribution to journalArticle

10 Scopus citations
16 Scopus citations
62 Scopus citations
2000

Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease

Ævarsson, A., Chuang, J. L., Max Wynn, R., Turley, S., Chuang, D. T. & Hol, W. G. J., Mar 1 2000, In : Structure. 8, 3, p. 277-291 15 p.

Research output: Contribution to journalArticle

112 Scopus citations
12 Scopus citations
18 Scopus citations
13 Scopus citations