J. Chuang

发表

Maple syrup urine disease in Mennonites. Evidence that the Y393N mutation in E1 alpha impedes assembly of the E1 component of branched-chain alpha-keto acid dehydrogenase complex.

C. W. Fisher, D. Chuang, R. Star, 1991, The Journal of clinical investigation.

Molecular basis of maple syrup urine disease: novel mutations at the E1 alpha locus that impair E1(alpha 2 beta 2) assembly or decrease steady-state E1 alpha mRNA levels of branched-chain alpha-keto acid dehydrogenase complex.

D. Chuang, C. Fisher, J. Chuang, 1994, American journal of human genetics.

Regional assignment of two genes of the human branched-chain α-keto acid dehydrogenase complex: The E1β gene (BCKDHB) to chromosome 6p21–22 and the E2 gene (DBT) to chromosome 1p31

R. Eddy, T. Shows, D. Chuang, 1991 .

Regional assignment of two genes of the human branched-chain alpha-keto acid dehydrogenase complex: the E1 beta gene (BCKDHB) to chromosome 6p21-22 and the E2 gene (DBT) to chromosome 1p31.

R. Eddy, T. Shows, D. Chuang, 1991, Genomics.

Structure-guided Development of Specific Pyruvate Dehydrogenase Kinase Inhibitors Targeting the ATP-binding Pocket*

D. Chuang, P. Scherer, N. Williams, 2013, The Journal of Biological Chemistry.

Pyruvate Dehydrogenase Kinase-4 Structures Reveal a Metastable Open Conformation Fostering Robust Core-free Basal Activity*

Jun Li, D. Chuang, Masato Kato, 2008, Journal of Biological Chemistry.

Structural Determinants for Cross-talk between Pyruvate Dehydrogenase Kinase 3 and Lipoyl Domain 2 of the Human Pyruvate Dehydrogenase Complex*

D. Chuang, Masato Kato, S. Tso, 2006, Journal of Biological Chemistry.

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

D. Chuang, Mingliang Lou, Cheng-Yang Wu, 2018, Molecular metabolism.

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

D. Chuang, B. Tu, Philippe M. Campeau, 2013, Proceedings of the National Academy of Sciences.

Novel Allosteric Inhibitors of Benzothiophene Carboxylate Derivatives as Metabolism

D. Chuang, S. Strom, K. Dorko, 2014 .

Benzothiophene Carboxylate Derivatives as Novel Allosteric Inhibitors of Branched-chain α-Ketoacid Dehydrogenase Kinase*

D. Chuang, S. Strom, K. Dorko, 2014, The Journal of Biological Chemistry.

Structural and Biochemical Characterization of Human Mitochondrial Branched-chain α-Ketoacid Dehydrogenase Phosphatase*

Jun Li, D. Chuang, C. Brautigam, 2012, The Journal of Biological Chemistry.

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

D. Chuang, R. Lebo, J. Chuang, 2000, American journal of human genetics.

Cloning and expression in Escherichia coli of mature E1 beta subunit of bovine mitochondrial branched-chain alpha-keto acid dehydrogenase complex. Mapping of the E1 beta-binding region on E2.

C. W. Fisher, D. Chuang, J. Davie, 1992, The Journal of biological chemistry.

Molecular Basis of Maple Syrup Urine Disease: Novel Mutations at the E I a Locus That Impair E I (a4,2) Assembly or Decrease Steady-State E I a mRNA Levels of Branched-Chain a-Keto Acid Dehydrogenase Complex

R. Fisher, J. Chuang, Cox, 2007 .

Secondary Effect of the Mutations

C. W. Fisher, D. Chuang, C. Fisher, 2006 .

Determination of protein complex stoichiometry through multisignal sedimentation velocity experiments.

D. Chuang, C. Brautigam, M. Rosen, 2010, Analytical biochemistry.

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

Jun Li, D. Chuang, M. Machius, 2004, Structure.

Tetrameric Assembly and Conservation in the ATP-binding Domain of Rat Branched-chain α-Ketoacid Dehydrogenase Kinase*

D. Chuang, J. Chuang, R. Wynn, 2000, The Journal of Biological Chemistry.

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

D. Chuang, A. Drousiotou, R. Wynn, 2009, Genetic testing and molecular biomarkers.

Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.

H. Mandel, D. Chuang, J. Chuang, 2001, The Journal of biological chemistry.

Impaired Assembly of E1 Decarboxylase of the Branched-chain α-Ketoacid Dehydrogenase Complex in Type IA Maple Syrup Urine Disease*

D. Chuang, J. Davie, J. Chuang, 1998, The Journal of Biological Chemistry.

Maple Syrup Urine Disease: Clinical and Therapeutic Considerations

D. Chuang, R. Wynn, J. Chuang, 2014 .

Lessons from genetic disorders of branched-chain amino acid metabolism.

D. Chuang, R. Wynn, J. Chuang, 2006, The Journal of nutrition.

Molecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patients.

D. Chuang, J. Davie, R. Wynn, 1995, The Journal of clinical investigation.

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

D. Chuang, Masato Kato, C. Brautigam, 2006, The EMBO journal.

Branched-Chain Amino Acids : Metabolism , Physiological Function , and Application Lessons from Genetic Disorders of Branched-Chain Amino Acid Metabolism

D. Chuang, R. Wynn, J. Chuang, 2005 .

Branched-Chain Amino Acids : Metabolism , Physiological Function , and Application Lessons from Genetic Disorders of Branched-Chain Amino Acid Metabolism 1 – 3

D. Chuang, R. Wynn, J. Chuang, 2005 .

Structural and Biochemical Basis for Novel Mutations in Homozygous Israeli Maple Syrup Urine Disease Patients

Jun Li, H. Mandel, D. Chuang, 2004, Journal of Biological Chemistry.

Three-Dimensional Structures for Components and Domain of the Mammalian Branched-Chain a-Ketoacid Dehydrogenase Complex

D. Chuang, R. Wynn, J. Chuang, 2003 .

Branched‐Chain α‐Keto Acid Dehydrogenase Complex, Human

D. Chuang, R. Wynn, J. Chuang, 2002 .

New Approaches to Detection and Characterization of PTH Receptors

J. Zull, J. Chuang, J. Reese, 1989 .

Specific cleavage of bovine parathyroid hormone catalyzed by an endopeptidase from bovine kidney.

B. Roos, A. Frelinger, J. Zull, 1981, The Journal of biological chemistry.

Metabolism of tritiated parathyroid hormone by a kidney cortex plasma membrane preparation.

J. Zull, J. Chuang, R. Botti, 1980, The Journal of biological chemistry.

Binding of tritiated bovine parathyroid hormone to plasma membranes from bovine kidney cortex.

C. Malbon, J. Zull, J. Chuang, 1977, The Journal of biological chemistry.

Expression of E1 component of human branched-chain alpha-keto acid dehydrogenase complex in Escherichia coli by cotransformation with chaperonins GroEL and GroES.

J. Song, D. Chuang, J. Davie, 2000, Methods in enzymology.

Maple Syrup Urine Disease in Cypriot Families: Identiﬁcation of Three Novel Mutations and Biochemical Characterization of the p.Thr211Met Mutation in the E1 a Subunit

D. Chuang, A. Drousiotou, T. Georgiou, 2010 .

E2 transacylase-deficient (type II) maple syrup urine disease. Aberrant splicing of E2 mRNA caused by internal intronic deletions and association with thiamine-responsive phenotype.

D. Chuang, J. Chuang, R. Cox, 1997, The Journal of clinical investigation.

Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene.

D. Chuang, D. Danner, J. Chuang, 1992, The Journal of biological chemistry.

Examination of parathyroid hormone antisera for the presence of receptor antibodies

J. Zull, J. Chuang, Susan Smith, 1989, Molecular and Cellular Endocrinology.

Structural and Biochemical Basis for Novel Mutations in Homozygous Israeli Maple Syrup Urine Disease Patients A Proposed Mechanism for Thiamin-responsive Phenotype*

Jun Li, H. Mandel, D. Chuang, 2003 .

Structural basis for inactivation of the human pyruvate dehydrogenase complex by phosphorylation: role of disordered phosphorylation loops.

Jun Li, Jun Li, D. Chuang, 2008, Structure.

Roles of Active Site and Novel K+ Ion-binding Site Residues in Human Mitochondrial Branched-chain α-Ketoacid Decarboxylase/Dehydrogenase*

D. Chuang, J. Chuang, R. Wynn, 2001, The Journal of Biological Chemistry.

The Two Active Sites in Human Branched-chain α-Keto Acid Dehydrogenase Operate Independently without an Obligatory Alternating-site Mechanism*

Jun Li, D. Chuang, M. Machius, 2007, Journal of Biological Chemistry.

Subunit and Catalytic Component Stoichiometries of an in Vitro Reconstituted Human Pyruvate Dehydrogenase Complex*

D. Chuang, C. Brautigam, R. Wynn, 2009, Journal of Biological Chemistry.

Characterization of the promoter-regulatory region and structural organization of E1α gene (BCKDHA) of human branched-chain α-keto acid dehydrogenase complex

D. Chuang, J. Chuang, R. Cox, 1993 .

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

Jun Li, Jun Li, D. Chuang, 2007, Structure.

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

Jun Li, Chad A Brautigam, Jun Li, 2006, Structure.

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

D. Chuang, M. Machius, D. Tomchick, 2005, Journal of molecular biology.

Production of recombinant mammalian holo-E2 and E3 and reconstitution of functional branched-chain alpha-keto acid dehydrogenase complex with recombinant E1.

D. Chuang, J. Davie, J. Chuang, 2000, Methods in enzymology.

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

Chad A Brautigam, D. Chuang, M. Machius, 2006, Structure.

Structural and Thermodynamic Basis for Weak Interactions between Dihydrolipoamide Dehydrogenase and Subunit-binding Domain of the Branched-chain α-Ketoacid Dehydrogenase Complex*

D. Chuang, C. Brautigam, Tai-Huang Huang, 2011, The Journal of Biological Chemistry.

Solution Structure and Dynamics of the Lipoic Acid-bearing Domain of Human Mitochondrial Branched-chain α-Keto Acid Dehydrogenase Complex*

Chi-Fon Chang, Hui-Ting Chou, Tai-huang Huang, 2002, The Journal of Biological Chemistry.

Identification and purification of a kidney membrane protein which specifically binds the amino-terminal domain of native parathyroid hormone.

J. Zull, J. Chuang, J. Reese, 1987, The Journal of biological chemistry.

GroEL/GroES-dependent Reconstitution of α2β2 Tetramers of Human Mitochondrial Branched Chain α-Ketoacid Decarboxylase

D. Chuang, J. Chuang, R. Wynn, 1999, The Journal of Biological Chemistry.

Structural Insight into Interactions between Dihydrolipoamide Dehydrogenase ( E 3 ) and E 3 Binding Protein of Human Pyruvate Dehydrogenase Complex

D. Chuang, M. Machius, D. Tomchick, 2022 .

Preparation and characterization of radioactive monoiodotyrosine and diiodotyrosine derivatives of parathyroid hormone.

J. Zull, J. Chuang, 1984, Analytical biochemistry.

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

D. Chuang, M. Machius, D. Tomchick, 2001, Proceedings of the National Academy of Sciences of the United States of America.

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

D. Chuang, Masato Kato, Jacinta L Chuang, 2005, The EMBO journal.

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

W. Hol, D. Chuang, S. Turley, 2000 .

Structure of the Subunit Binding Domain and Dynamics of the Di-domain Region from the Core of Human Branched Chain α-Ketoacid Dehydrogenase Complex*

D. Chuang, Tai-Huang Huang, J. Chuang, 2006, Journal of Biological Chemistry.

Roles of His291-α and His146-β′ in the Reductive Acylation Reaction Catalyzed by Human Branched-chain α-Ketoacid Dehydrogenase

Jun Li, Jun Li, D. Chuang, 2003, Journal of Biological Chemistry.

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.

Jun Li, Jun Li, D. Chuang, 2003, The Journal of biological chemistry.

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

W. Hol, D. Chuang, S. Turley, 2000, Structure.

Structural and Biochemical Characterization of Human Mitochondrial Branched-chain -Ketoacid Dehydrogenase

Jun Li, D. Chuang, C. Brautigam, 2012 .

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

D. Chuang, Mingliang Lou, N. Williams, 2017, Journal of medicinal chemistry.

Molecular Phenotypes in Cultured Maple Syrup Urine Disease Cells

C. W. Fisher, D. Chuang, T. A. Griffin, 1989, The Journal of biological chemistry.

Kidney membrane binding of native parathyroid hormone compared to binding of its synthetic 1 - 34 fragment.

J. Zull, J. Chuang, 1980, Journal of receptor research.

Molecular basis of maple syrup urine disease and stable correction by retroviral gene transfer.

D. Chuang, J. Davie, J. Chuang, 1995, The Journal of nutrition.

Occurrence of a 2-bp (AT) deletion allele and a nonsense (G-to-T) mutant allele at the E2 (DBT) locus of six patients with maple syrup urine disease: multiple-exon skipping as a secondary effect of the mutations.

C. W. Fisher, D. Chuang, C. Fisher, 1993, American journal of human genetics.

The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain alpha-keto acid dehydrogenase complex.

D. Chuang, D. Danner, J. Chuang, 1992, Biochimica et biophysica acta.

Cross-talk between Thiamin Diphosphate Binding and Phosphorylation Loop Conformation in Human Branched-chain α-Keto Acid Decarboxylase/Dehydrogenase*

Jun Li, D. Chuang, M. Machius, 2004, Journal of Biological Chemistry.

The C-terminal Hinge Region of Lipoic Acid-bearing Domain of E2b Is Essential for Domain Interaction with Branched-chain α-Keto Acid Dehydrogenase Kinase*

D. Chuang, R. Wynn, J. Chuang, 2002, The Journal of Biological Chemistry.

Determination of protein complex stoichiometry through multisignal sedimentation velocity experiments.

D. Chuang, C. Brautigam, S. Padrick, 2010, Analytical biochemistry.

Diagnosis and mutational analysis of maple syrup urine disease using cell cultures.

J. Chuang, David T. Chuang, 2000, Methods in Enzymology.

J. Chuang, David T. Chuang, R. P. Cox, 1994, American journal of human genetics.