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

Wynn R.M., Li J., Brautigam C.A., Chuang J.L., Chuang D.T.

J. Biol. Chem. 287:9178-9192(2012) · Mapped (1)

Structural and thermodynamic basis for weak interactions between dihydrolipoamide dehydrogenase and subunit-binding domain of the branched-chain alpha-ketoacid dehydrogenase complex.

Brautigam C.A., Wynn R.M., Chuang J.L., Naik M.T., Young B.B., Huang T.H., Chuang D.T.

J. Biol. Chem. 286:23476-23488(2011) · Mapped (6)

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.

J. Biol. Chem. 285:265-276(2010) · Mapped (5)

Pivotal role of the C-terminal DW-motif in mediating inhibition of pyruvate dehydrogenase kinase 2 by dichloroacetate.

Li J., Kato M., Chuang D.T.

J. Biol. Chem. 284:34458-34467(2009) · UniProtKB (1) · Mapped (2)

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.

Genet Test Mol Biomarkers 13:657-664(2009) · Mapped (6)

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.

Structure 16:1849-1859(2008) · UniProtKB (2)

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.

J. Biol. Chem. 283:25305-25315(2008) · UniProtKB (1) · Mapped (4)

De novo backbone trace of GroEL from single particle electron cryomicroscopy.

Ludtke S.J., Baker M.L., Chen D.H., Song J.L., Chuang D.T., Chiu W.

Structure 16:441-448(2008) · Mapped (2)

Distinct structural mechanisms for inhibition of pyruvate dehydrogenase kinase isoforms by AZD7545, dichloroacetate, and radicicol.

Kato M., Li J., Chuang J.L., Chuang D.T.

Structure 15:992-1004(2007) · UniProtKB (3) · Mapped (5)

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

Li J., Machius M., Chuang J.L., Wynn R.M., Chuang D.T.

J. Biol. Chem. 282:11904-11913(2007) · Mapped (7)

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

Kato M., Wynn R.M., Chuang J.L., Brautigam C.A., Custorio M., Chuang D.T.

EMBO J. 25:5983-5994(2006) · UniProtKB (1)

Structure of the subunit binding domain and dynamics of the di-domain region from the core of human branched chain alpha-ketoacid dehydrogenase complex.

Chang C.F., Chou H.T., Lin Y.J., Lee S.J., Chuang J.L., Chuang D.T., Huang T.H.

J. Biol. Chem. 281:28345-28353(2006) · Mapped (3)

Structural determinants for cross-talk between pyruvate dehydrogenase kinase 3 and lipoyl domain 2 of the human pyruvate dehydrogenase complex.

Tso S.C., Kato M., Chuang J.L., Chuang D.T.

J. Biol. Chem. 281:27197-27204(2006) · Mapped (1)

A versatile conformational switch regulates reactivity in human branched-chain alpha-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.

Structure 14:287-298(2006) · Mapped (7)

Structural insight into interactions between dihydrolipoamide dehydrogenase (E3) and E3 binding protein of human pyruvate dehydrogenase complex.

Brautigam C.A., Wynn R.M., Chuang J.L., Machius M., Tomchick D.R., Chuang D.T.

Structure 14:611-621(2006) · UniProtKB (2) · Mapped (6)

Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations.

Brautigam C.A., Chuang J.L., Tomchick D.R., Machius M., Chuang D.T.

J. Mol. Biol. 350:543-552(2005) · UniProtKB (1) · Mapped (5)

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.

EMBO J. 24:1763-1774(2005) · UniProtKB (2)

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

Wynn R.M., Kato M., Machius M., Chuang J.L., Li J., Tomchick D.R., Chuang D.T.

Structure 12:2185-2196(2004) · Mapped (2)

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

Li J., Wynn R.M., Machius M., Chuang J.L., Karthikeyan S., Tomchick D.R., Chuang D.T.

J. Biol. Chem. 279:32968-32978(2004) · Mapped (7)

Roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site.

Wynn R.M., Machius M., Chuang J.L., Li J., Tomchick D.R., Chuang D.T.

J. Biol. Chem. 278:43402-43410(2003) · Mapped (9)

Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex.

Chang C.-F., Chou H.-T., Chuang J.L., Chuang D.T., Huang T.-H.

J. Biol. Chem. 277:15865-15873(2002) · UniProtKB (1) · Mapped (4)

Structure of rat BCKD kinase: nucleotide-induced domain communication in a mitochondrial protein kinase.

Machius M., Chuang J.L., Wynn R.M., Tomchick D.R., Chuang D.T.

Proc. Natl. Acad. Sci. U.S.A. 98:11218-11223(2001) · Mapped (1)

Biochemical basis of type IB (E1beta ) 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.

J. Biol. Chem. 276:36550-36556(2001) · Mapped (3)

Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.

Wynn R.M., Ho R., Chuang J.L., Chuang D.T.

J. Biol. Chem. 276:4168-4174(2001) · Mapped (2)

Rare etiology of autosomal recessive disease in a child with noncarrier parents.

Lebo R.V., Shapiro L.R., Fenerci E.Y., Hoover J.M., Chuang J.L., Chuang D.T., Kronn D.F.

Am. J. Hum. Genet. 67:750-754(2000) · Mapped (2)

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