RodyPCoxMD

PubMed

• 7 citations
  A distinct variant of intermediate maple syrup urine disease.
  Maria del Carmen Gonzalez-Rios, David T. Chuang, Rody P. Cox, Kathleen Schmidt, Karen Knopf, Seymour Packman> ;Clinical Genetics. 2008 Apr 23
• 63 citations
  Identification of the α-Aminoadipic Semialdehyde Synthase Gene, Which Is Defective in Familial Hyperlysinemia
  Katherine A. Sacksteder, Barbara J. Biery, James C. Morrell, Barbara K. Goodman, Brian V. Geisbrecht, Rody P. Cox, Stephen Jay Gould, Michael T. Geraghty> ;American Journal of Human Genetics. 2000 Jun 1
• 19 citations
  E2 transacylase-deficient (type II) maple syrup urine disease. Aberrant splicing of E2 mRNA caused by internal intronic deletions and association with thiamine-respons...
  Jacinta L. Chuang, Rody P. Cox, David T. Chuang> ;The Journal of Clinical Investigation. 1997 Aug 1
• 3 citations
  Maple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E...
  Jacinta L. Chuang, Rody P. Cox, David T. Chuang> ;American Journal of Human Genetics. 1996 Jun 1
• 4 citations
  Molecular basis of maple syrup urine disease and stable correction by retroviral gene transfer.
  David T. Chuang, James R. Davie, Richard M Wynn, Jacinta L. Chuang, H. Koyata, Rody P. Cox> ;The Journal of Nutrition. 1995 Jun 1
• 25 citations
  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...
  Jacinta L. Chuang, James R. Davie, J M Chinsky, R M Wynn, Rody P. Cox, David T. Chuang> ;The Journal of Clinical Investigation. 1995 Mar 1
• 2 citations
  Differential processing of human and rat E1 α precursors of the branched-chain α-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence
  Richard M Wynn, Hideo Kochi, Rody P. Cox, David T. Chuang> ;Biochimica Et Biophysica Acta. 1994 Sep 28
• 13 citations
  In vitro Reconstitution of the 24-meric E2 Inner Core of Bovine Mitochondrial Branched-Chain .alpha.-Keto Acid Dehydrogenase Complex: Requirement for Chaperonins GroEL...
  R. Max Wynn, James R. Davie, Wang Zhi, Rody P. Cox, David T. Chuang> ;Biochemistry. 1994 Aug 2
• 48 citations
  Molecular chaperones: Heat-shock proteins, foldases, and matchmakers
  R. Max Wynn, James R. Davie, Rody P. Cox, David T. Chuang> ;The Journal of Laboratory and Clinical Medicine. 1994 Jul 1
• 12 citations
  Stable correction of maple syrup urine disease in cells from a Mennonite patient by retroviral-mediated gene transfer.
  H. Koyata, Rody P. Cox, David T. Chuang> ;The Biochemical Journal. 1993 Nov 1
• 14 citations
  Characterization of the promoter-regulatory region and structural organization of E1 alpha gene (BCKDHA) of human branched-chain alpha-keto acid dehydrogenase complex.
  Jacinta L. Chuang, Rody P. Cox, David T. Chuang> ;The Journal of Biological Chemistry. 1993 Apr 15
• 42 citations
  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 ski...
  Charles W. Fisher, Carolyn R. Fisher, Jacinta L. Chuang, Kim S. Lau, David T. Chuang, Rody P. Cox> ;American Journal of Human Genetics. 1993 Feb 1
• 21 citations
  Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 ps...
  Kim S. Lau, W J Herring, Jacinta L. Chuang, M McKean, D J Danner, Rody P. Cox, David T. Chuang> ;The Journal of Biological Chemistry. 1992 Nov 25
• 17 citations
  The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain α-keto acid dehydrogenase complex
  Kim S. Lau, Jacinta L. Chuang, W. Joseph Herring, Dean J. Danner, Rody P. Cox, David T. Chuang> ;Biochimica Et Biophysica Acta. 1992 Oct 20
• 30 citations
  Expression and assembly of a functional E1 component (alpha 2 beta 2) of mammalian branched-chain alpha-ketoacid dehydrogenase complex in Escherichia coli.
  James R. Davie, Richard M Wynn, Rody P. Cox, David T. Chuang> ;The Journal of Biological Chemistry. 1992 Aug 15
• 62 citations
  Chaperonins groEL and groES promote assembly of heterotetramers (alpha 2 beta 2) of mammalian mitochondrial branched-chain alpha-keto acid decarboxylase in Escherichia...
  Richard M Wynn, James R. Davie, Rody P. Cox, David T. Chuang> ;The Journal of Biological Chemistry. 1992 Jun 25
• 27 citations
  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 be...
  Richard M Wynn, Jacinta L. Chuang, James R. Davie, Charles W. Fisher, Michael A. Hale, Rody P. Cox, David T. Chuang> ;The Journal of Biological Chemistry. 1992 Jan 25
• 3 citations
  Structure of the gene encoding the entire mature E1 alpha subunit of human branched-chain alpha-keto acid dehydrogenase complex (1991) FEBS Letters 284, 34-38.
  N. Dariush, Charles W. Fisher, Rody P. Cox, David T. Chuang> ;FEBS Letters. 1991 Oct 21
• 29 citations
  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 dehydrogen...
  Carolyn R. Fisher, Jacinta L. Chuang, Rody P. Cox, Charles W. Fisher, Robert A. Star, David T. Chuang> ;The Journal of Clinical Investigation. 1991 Sep 1
• 30 citations
  Occurrence of a Tyr393----Asn (Y393N) mutation in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients f...
  Carolyn R. Fisher, Charles W. Fisher, David T. Chuang, Rody P. Cox> ;American Journal of Human Genetics. 1991 Aug 1
• 9 citations
  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 ch...
  Susan M. Zneimer, Kim S. Lau, Roger L. Eddy, Thomas B. Shows, Jacinta L. Chuang, David T. Chuang, Rody P. Cox> ;Genomics. 1991 Jul 1
• 13 citations
  Structure of the gene encoding the entire mature E1α subunit of human branched-chain α-keto acid dehydrogenase complex
  Nahid Dariush, Charles W. Fisher, Rody P. Cox, David T. Chuang> ;FEBS Letters. 1991 Jun 17
• 4 citations
  Premature termination of transcription and alternative splicing in the human transacylase (E2) gene of the branched-chain α-ketoacid dehydrogenase complex
  Kim S. Lau, Jun Lee, Charles W. Fisher, Rody P. Cox, David T. Chuang> ;FEBS Letters. 1991 Feb 25
• 11 citations
  Maple syrup urine disease: Domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain α-keto acid dehydrogena...
  David T. Chuang, Charles W. Fisher, Kim S. Lau, Thomas A. Griffin, Richard M Wynn, Rody P. Cox> ;Molecular Biology & Medicine. 1991 Feb 1
• 24 citations
  A 17-bp insertion and a Phe215----Cys missense mutation in the dihydrolipoyl transacylase (E2) mRNA from a thiamine-responsive maple syrup urine disease patient WG-34.
  Charles W. Fisher, Kim S. Lau, Carolyn R. Fisher, R. Max Wynn, Rody P. Cox, David T. Chuang> ;Biochemical and Biophysical Research Communications. 1991 Jan 31
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Journal Articles

• Identification of the alpha aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia  
  Sacksteder KA, Biery BJ, Morrell JC, Goodman BK, Cox RP, Gould SJ, Geraghty MT, Am J Hum Genet, 1/1/2000
• Characterization of the gene encoding alpha-aminoadipic semialdehyde synthase  
  Sacksteder KA, Biery BJ, Morrell JC, Geisbrecht BV, Cox RP, Gould SJ, Geraghty MT, Am J Hum Genet, 1/1/1999
• Impaired assembly of E1 decarboxylase of the branched-chain alpha-ketoacid dehydrogenase complex in type 1A maple syrup urine disease  
  Wynn RM, Davie JR, Chuang JL, Cote CD, Cox R, Chuang DT, J Biol Chem, 1/1/1998
• Molecular genetic basis of Type II maple syrup urine disease Novel splicing errors caused by deletions of internal intronic segments in E2 gene and correlations with ...  
  Chuang JL, Cox RP, Chuang DT, J Clin Invest, 1/1/1997
• The E1beta gene of human branched-chain alpha ketoacid dehydrogenase complex has 11 rather than 10 exons and the 3’ UTR in one of the two E1beta mRNA arises from intro...  
  Chuang JL, Cox RP, Chuang DT, Am J Hum Genet, 1/1/1996
• Molecular basis of maple syrup urine disease and stable correction by retroviral gene transfer  
  Chuang DT, Davie JR, Wynn RM, Chuang JL, Koyata H, Cox RP, J Nutrition, 1/1/1995
• Molecular and biochemical basis of intermediate maple syrup urine disease Occurrence of homozygous G245R and F364C mutations at the E1alpha locus of Hispanic-Mexican ...  
  Chuang JL, Davie JR, Chinsky JM, Wynn RM, Cox RP, Chuang DT, J Clin Invest, 1/1/1995
• Molecular basis of maple syrup urine disease: Novel mutations of the E1 locus that impair E1(alpa2beta2) assembly or decrease steady-state E1alpha mRNA levels of the b...  
  Chuang JL, Fisher CR, Cox RP, Chuang DT, Am J Hum Genet, 1/1/1994
• Chaperonins GroEL/ES mediate in vitro refolding and assembly of E2 inner-core 24-mers of bovine mitochondrial branched chain alpha-keto acid dehydrogenase complex  
  Wynn RM, Davie JR, Zhi W, Cox RP, Chuang DT, Biochemistry, 1/1/1994
• Molecular chaperones Heat shock proteins, foldases and matchmakers  
  Wynn RN, Davie JR, Cox RP, Chuang DT, J Lab Clin Med, 1/1/1994
• Differential processing of human and rat E1alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rate seq...  
  Wynn RM, Kochi H, Cox RP, Chuang DT, Biochem Biophys Acta, 1/1/1994
• Occurrence of a two base pair (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 ...  
  Fisher CW, Fisher CR, Chuang JL, Lau KS, Chuang DT, Cox RP, Am J Hum Genet, 1/1/1993
• Characterization of the promoter-regulatory region and structural organization of E1alpha gene (BCKAHA) of human branched-chain alpha-keto acid dehydrogenase complex  
  Chuang JL, Cox RP, Chuang DT, J Biol Chem, 1/1/1993
• Stable correction of maple syrup urine disease in cells from a Mennonite patient by retroviral-mediated gene transfer  
  Koyata H, Cox RP, Chuang DT, Biochem J, 1/1/1993
• Cloning and expression in Escherihia coli of mature E1 subunit of bovine mitochondrial branched-chain alpha-keto acid dehydrogenase complex: Mapping of E1alpha-binding...  
  Wynn RM Chuang JL, Davie JR, Fisher CW, Hale MA, Cox RP, Chuang DT, J Biol Chem, 1/1/1992
• Chaperonins GroEL and GroES promote assembly of heterotramers (alpha2beta2) of mammalian mitochondrial branched-chain alpha-keto acid decarboxylase in Escherichia coli  
  Wynn RM, Davie JR, Cox RP, Chuang DT, J Biol Chem, 1/1/1992
• Espression and assembly of a functional E1 component (alpha2beta2) of mammalian branched-chain alpha-keto acid dehydrogenase complex in Escherichia coli  
  Davie JR, Wynn RM, Cox RP, Chuang DT, J Biol Chem, 1/1/1992
• Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched-chain alpha-keto acid dehydrogenase complex and characterization of an E2 ps...  
  Lau KS, Herring WJ, Chuang JL, McKean M, Danner DJ, Cox RP, Chuang DT, J Biol Chem, 1/1/1992
• The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain alpha-keto acid dehydrogenase complex  
  Lau KS, Chuang JL, Herring WJ, Danner DJ, Cox RP, Chuang DT, Biochim Biophy Acta, 1/1/1992
• Premature termination of transcription and alternative splicing in the human transacylase (E2) gene of the branched-chain alpha-keto acid dehydrogenase complex  
  Lau KS, Lee J, Fisher CW, Cox RP, Chuang DT, FEBS Lett, 1/1/1991
• Localization of the dihydrolipoamide branched-chain transacylase gene (DBT) of the human branched-chain keto acid dehydrogenase complex to chromosome 1  
  Lau KS, Eddy RL, Shows TB, Fisher CW, Chuang DT, Cox RP, Cytogenet Cell Genet, 1/1/1991
• A 17-bp insertion and a Phe215 Cys missense mutation in the dihydrolipoyl transacylase (E2) mRNA from a thiamine-responsive maple syrup urine disease patient WG-34  
  Fisher CW, Lau KS, Fisher CR, Wynn RM, Cox RP, Chuang DT, Biochem Biophy Res Comm, 1/1/1991
• Regional assignment of two genes of the human branched-chain alpha-keto acid dehydrogenase complex: The E1alpha gene (BCKDHB) to chromosome 6p21-22 and the E2 gene (DB...  
  Zneimer SM, Lau KS, Eddy RL, Shows TB, Chuang DT, Cox RP, Genomics, 1/1/1991
• Maple syrup urine disease: Domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydro...  
  Chuang DT, Fisher CW, Lau KS, Griffin TA, Wynn RM, Cox RP, Mol Biol Med, 1/1/1991
• Occurrence of a Tyr 393 Asn (Y393N) mutation in the E1alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients from...  
  Fisher CR, Fisher CW, Chuang DT, Cox RP, Am J Hum Genet, 1/1/1991
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Books/Book Chapters

• The Molecular and Genetic Basis of Neurologic and Psychiatric Diseases 
  Chapter: Maple Syrup Urine Disease: Clinical and Therapeutic Considerations 
  Page 663.0 - 672.0Chuang DT, Wynn RM, Cox RP and Chuang JLAcademic Press, New YorkEdition 4th,2015
• The Metabolic and Molecular Basis of Inherited Disease 
  Chapter: Errors of lysine metabolism 
  Cox RP, Geraghty MG2012
• The Molecular and Genetic Basis of Neurologic and Psychiatric Disease 
  Chapter: Maple syrup urine disease: Clinical and biochemical perspectives 
  Page 635.0 - 642.0Chuang DT, Chuang JL, Cox RPEditors: RN Rosenberg, SB Prusiner, S DiMauro, RL Barchi, EJ Nestler.Butterworth and Heinemann, PhiladelphiaEdition 3rd,2003
• The Metabolic and Molecular Basis of Inherited Diseases 
  Chapter: Lysine metabolism 
  Page 1965. - 1970.Cox RPEditors: CR Scriver, AL Beaudet, WS Sly, D Valle.Edition 8th,2000
• The Metabolic Basis of Inherited Disease 
  Chapter: Errors of lysine metabolism 
  Page 1233. - 1238.Cox RP, Dancis JEditors: AL Beaudet, WS Sly, D Valle.McGraw-HillEdition 7th,1995
• The Molecular and Genetic Basis of Neurologic Disease 
  Chapter: Maple syrup urine disease: Clinical and molecular genetic considerations 
  Page 189.0 - 207.0Cox RP, Chuang DTEditors: RN Rosenberg, SB Prusiener, S DiMauro, RL Barchi, LM Kunkel.Butterworth-Heinemann, BostonEdition 1st,1993
• The Molecular and Genetic Basis of Neurological Disease 
  Chapter: Maple syrup urine disease: Clinical and molecular considerations 
  Page 189.0 - 207.0Cox RP, Chuang DTEditors: RN Rosenberg, SB Prusiner, S DiMauro, RL Barchi, LM Kunkel.Butterworths Publ, London1992
• The Metabolic Basis of Inherited Disease 
  Chapter: Lysine metabolism 
  Page 665.0 - 670.0Dancis J, Cox RPEditors: CR Scriver, AL Beaudet, WS Sly, D Valle.McGraw Hill Book Co, NYEdition 6th,1989
• Congenital Metabolic Diseases 
  Chapter: Disease 
  Page 375.0 - 384.0Dancis J, Cox RP, Balis EM, Lesch-NyhanEditors: RA Wapnir.Dekker Press, NY1985
• Epithelial Cell Cultures 
  Chapter: Morphologic and biosynthetic modulation of HeLa cells mediated by sodium butyrate 
  Page 129.0 - 143.0Fallon RJ, Ghosh NK, Cox RPEditors: BS Danes, RP Cox.1981
• Advances in Cell Culture 
  Chapter: Glycopeptide hormone production in cell culture 
  Page 15.0 - 66.0Cox RP, Day DGEditors: K Maramorosch.Academic Press, NY1981
• Proceedings of Second International Conference on Diagnosis and Treatment of Craniofacial Anomalies 
  Chapter: Genetic principles and practices applied to craniofacial malformations 
  Page 54.0 - 67.0Cox RP, Reich EWEditors: JM Converse, J McCarthy.CV Mosby, St. Louis1979
• Calcium Binding Proteins and Calcium Function 
  Chapter: Calcium-mediated regulation of intracellular human chorionic gonadotropin in HeLa65 cell cultures 
  Page 495.0 - 497.0Fallon RJ, Ghosh NK, Cox RPEditors: RH Wasserman, et al.Elsevier North-Holland Inc Publishers, NY1977
• Trends and Teaching in Clinical Genetics 
  Chapter: Interdisciplinary education in clinical genetics 
  Page 155.0 - 165.0Cox RP, Reich EW, Cox JBAlan R Liss, Inc, NY1977
• Cancer of the Skin 
  Chapter: Genetics of Cancer 
  Page 137.0 - 162.0Cox RPEditors: R Andrade, SL Gumport, GL Popkin, TD Rees.WB Saunders, Philadelphia1976
• Isozymes, Vol 1 Molecular Structure 
  Chapter: Hormonally induced modification of HeLa alkaline phosphatase with increased catalytic activity 
  Page 343.0 - 366.0Cox RP, Ghosh NK, Bazzell, KL, Griffin MJEditors: CL Markert.Academic Press, New York1975
• Purine Metabolism in Man 
  Chapter: Unstable HPRTase in subjects with abnormal oxypurine excretion 
  Page 195.0 - 202.0Balis ME, Yip LC, Yu TF, Gutman AB, Cox RP, Dancis JEditors: A DeVries, J Wyngaarden.1974
• Methods in Tissue Culture 
  Chapter: Evaluation of amino acid metabolism in maple syrup urine disease 
  Page 639.0 - 643.0Dancis J, Hutzler J, Cox RPEditors: P Kruse.Academic Press, NY1973
• Regulation of Gene Expression in Cultured Cells 
  Chapter: Steroid control of the activity of metalloenzymes in eukaryotic cells 
  Page 77.0 - 82.0Cox RPEditors: M Harris.1973
• Proceedings of the Third Josiah Macy Conference on Genetics 
  Chapter: Rare genes and their relation to host susceptibility to infectious diseases 
  Page 219.0 - 232.0Cox RPEditors: WJ Schull.1963
• Methodology in Human Genetics 
  Chapter: Relation of genetic abnormalities of man to susceptibility to infectious diseases 
  Cox RP, MacLeod CMEditors: WJ Burdette.
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Lectures

• Reflections on Medical Education in the 1940’s and the Conquest of Disease 
  UT Southwestern Medical Center - 1/1/2012
• Polyglutamines and Neurodegenerative Diseases 
  UT Southwestern Medical Center - 1/1/2004
• DNA Methylation: A Two-Edged Sword for Embryology and Cancer 
  UT Southwestern Medical Center - 1/1/2001
• Amyloidosis – Diseases of Misfolded Proteins 
  UT Southwestern Medical Center - 1/1/1998
• Genetics of Obesity 
  UT Southwestern Medical Center - 1/1/1996
• The Genome Project – Medical, Social, Ethical and Legal Implications 
  UT Southwestern Medical Center - 1/1/1993
• Medical Genetics for Internists Homocystinuria and Marfan Syndrome 
  UT Southwestern Medical Center - 1/1/1991
• AOA Lecture 
  Case Western Reserve Union - 1/1/1990
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