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Institution

Tuchman, Mendel

TitleScientific Director
InstitutionChildrens National Medical Center
DepartmentCRI Administration
Address111 Michigan Ave NW
Washington DC 20010
Phone(202) 4762549
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    Collapse Bibliographic 
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    1. Shi D, Zhao G, Ah Mew N, Tuchman M. Precision medicine in rare disease: Mechanisms of disparate effects of N-carbamyl-l-glutamate on mutant CPS1 enzymes. Mol Genet Metab. 2017 Mar; 120(3):198-206. PMID: 28007335.
      View in: PubMed
    2. Dercksen M, Duran M, IJlst L, Kulik W, Ruiter JP, van Cruchten A, Tuchman M, Wanders RJ. A novel UPLC-MS/MS based method to determine the activity of N-acetylglutamate synthase in liver tissue. Mol Genet Metab. 2016 Dec; 119(4):307-310. PMID: 27771289.
      View in: PubMed
    3. Shi D, Allewell NM, Tuchman M. From Genome to Structure and Back Again: A Family Portrait of the Transcarbamylases. Int J Mol Sci. 2015 Aug 12; 16(8):18836-64. PMID: 26274952; PMCID: PMC4581275.
    4. Shi D, Allewell NM, Tuchman M. The N-Acetylglutamate Synthase Family: Structures, Function and Mechanisms. Int J Mol Sci. 2015 Jun 09; 16(6):13004-22. PMID: 26068232; PMCID: PMC4490483.
    5. Caldovic L, Abdikarim I, Narain S, Tuchman M, Morizono H. Genotype-Phenotype Correlations in Ornithine Transcarbamylase Deficiency: A Mutation Update. J Genet Genomics. 2015 May 20; 42(5):181-94. PMID: 26059767; PMCID: PMC4565140.
    6. Zhao G, Jin Z, Allewell NM, Tuchman M, Shi D. Structures of the N-acetyltransferase domain of Xylella fastidiosa N-acetyl-L-glutamate synthase/kinase with and without a His tag bound to N-acetyl-L-glutamate. Acta Crystallogr F Struct Biol Commun. 2015 Jan 01; 71(Pt 1):86-95. PMID: 25615976; PMCID: PMC4304755.
    7. Batshaw ML, Tuchman M, Summar M, Seminara J. A longitudinal study of urea cycle disorders. Mol Genet Metab. 2014 Sep-Oct; 113(1-2):127-30. PMID: 25135652; PMCID: PMC4178008.
    8. Ah Mew N, McCarter R, Daikhin Y, Lichter-Konecki U, Nissim I, Yudkoff M, Tuchman M. Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate. J Pediatr. 2014 Aug; 165(2):401-403.e3. PMID: 24880889; PMCID: PMC4111993.
    9. Caldovic L, Haskins N, Mumo A, Majumdar H, Pinter M, Tuchman M, Krufka A. Expression pattern and biochemical properties of zebrafish N-acetylglutamate synthase. PLoS One. 2014; 9(1):e85597. PMID: 24465614; PMCID: PMC3899043.
    10. Mew NA, Yudkoff M, Tuchman M. Stable isotopes in the diagnosis and treatment of inherited hyperammonemia. J Pediatr Biochem. 2014 Jan 01; 4(1):57-63. PMID: 24634704.
      View in: PubMed
    11. Zhao G, Jin Z, Allewell NM, Tuchman M, Shi D. Crystal structure of the N-acetyltransferase domain of human N-acetyl-L-glutamate synthase in complex with N-acetyl-L-glutamate provides insights into its catalytic and regulatory mechanisms. PLoS One. 2013; 8(7):e70369. PMID: 23894642; PMCID: PMC3722096.
    12. Zhao G, Jin Z, Wang Y, Allewell NM, Tuchman M, Shi D. Structure and function of Escherichia coli RimK, an ATP-grasp fold, L-glutamyl ligase enzyme. Proteins. 2013 Oct; 81(10):1847-54. PMID: 23609986.
      View in: PubMed
    13. Zhao G, Haskins N, Jin Z, M Allewell N, Tuchman M, Shi D. Structure of N-acetyl-L-glutamate synthase/kinase from Maricaulis maris with the allosteric inhibitor L-arginine bound. Biochem Biophys Res Commun. 2013 Aug 09; 437(4):585-90. PMID: 23850694; PMCID: PMC3754781.
    14. Zhao G, Allewell NM, Tuchman M, Shi D. Structure of the complex of Neisseria gonorrhoeae N-acetyl-L-glutamate synthase with a bound bisubstrate analog. Biochem Biophys Res Commun. 2013 Jan 25; 430(4):1253-8. PMID: 23261468; PMCID: PMC3558559.
    15. Ah Mew N, Krivitzky L, McCarter R, Batshaw M, Tuchman M. Clinical outcomes of neonatal onset proximal versus distal urea cycle disorders do not differ. J Pediatr. 2013 Feb; 162(2):324-9.e1. PMID: 22901741; PMCID: PMC4440324.
    16. Preparedness of the CTSA's structural and scientific assets to support the mission of the National Center for Advancing Translational Sciences (NCATS). Clin Transl Sci. 2012 Apr; 5(2):121-9. PMID: 22507116; PMCID: PMC3335735.
    17. Senkevitch E, Cabrera-Luque J, Morizono H, Caldovic L, Tuchman M. A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase. Mol Genet Metab. 2012 Jun; 106(2):160-8. PMID: 22503289; PMCID: PMC3356441.
    18. Heibel SK, Lopez GY, Panglao M, Sodha S, Mariño-Ramírez L, Tuchman M, Caldovic L. Transcriptional regulation of N-acetylglutamate synthase. PLoS One. 2012; 7(2):e29527. PMID: 22383952; PMCID: PMC3287996.
    19. Shi D, Yu X, Zhao G, Ho J, Lu S, Allewell NM, Tuchman M. Crystal structure and biochemical properties of putrescine carbamoyltransferase from Enterococcus faecalis: Assembly, active site, and allosteric regulation. Proteins. 2012 May; 80(5):1436-47. PMID: 22328207; PMCID: PMC3526856.
    20. Joseph J, Purucker M, Eskandanian K, Batshaw M, Tuchman M. The Clinical and Translational Science Institute at Children's National: improving health through pediatric research. Clin Transl Sci. 2012 Feb; 5(1):5-7. PMID: 22376250.
      View in: PubMed
    21. Shi D, Li Y, Cabrera-Luque J, Jin Z, Yu X, Zhao G, Haskins N, Allewell NM, Tuchman M. A novel N-acetylglutamate synthase architecture revealed by the crystal structure of the bifunctional enzyme from Maricaulis maris. PLoS One. 2011; 6(12):e28825. PMID: 22174908; PMCID: PMC3236213.
    22. Heibel SK, Ah Mew N, Caldovic L, Daikhin Y, Yudkoff M, Tuchman M. N-carbamylglutamate enhancement of ureagenesis leads to discovery of a novel deleterious mutation in a newly defined enhancer of the NAGS gene and to effective therapy. Hum Mutat. 2011 Oct; 32(10):1153-60. PMID: 21681857; PMCID: PMC3976964.
    23. Li Y, Jin Z, Yu X, Allewell NM, Tuchman M, Shi D. The ygeW encoded protein from Escherichia coli is a knotted ancestral catabolic transcarbamylase. Proteins. 2011 Jul; 79(7):2327-34. PMID: 21557323; PMCID: PMC3115391.
    24. Nissim I, Horyn O, Nissim I, Daikhin Y, Caldovic L, Barcelona B, Cervera J, Tuchman M, Yudkoff M. Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase. J Biol Chem. 2011 Jun 24; 286(25):22055-68. PMID: 21540182; PMCID: PMC3121350.
    25. Li Y, Yu X, Ho J, Fushman D, Allewell NM, Tuchman M, Shi D. Reversible post-translational carboxylation modulates the enzymatic activity of N-acetyl-L-ornithine transcarbamylase. Biochemistry. 2010 Aug 17; 49(32):6887-95. PMID: 20695527; PMCID: PMC2959125.
    26. Ah Mew N, McCarter R, Daikhin Y, Nissim I, Yudkoff M, Tuchman M. N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. Pediatrics. 2010 Jul; 126(1):e208-14. PMID: 20566609; PMCID: PMC3297024.
    27. Caldovic L, Ah Mew N, Shi D, Morizono H, Yudkoff M, Tuchman M. N-acetylglutamate synthase: structure, function and defects. Mol Genet Metab. 2010; 100 Suppl 1:S13-9. PMID: 20303810; PMCID: PMC2876818.
    28. Yudkoff M, Ah Mew N, Daikhin Y, Horyn O, Nissim I, Nissim I, Payan I, Tuchman M. Measuring in vivo ureagenesis with stable isotopes. Mol Genet Metab. 2010; 100 Suppl 1:S37-41. PMID: 20338795; PMCID: PMC2858793.
    29. Seminara J, Tuchman M, Krivitzky L, Krischer J, Lee HS, Lemons C, Baumgartner M, Cederbaum S, Diaz GA, Feigenbaum A, Gallagher RC, Harding CO, Kerr DS, Lanpher B, Lee B, Lichter-Konecki U, McCandless SE, Merritt JL, Oster-Granite ML, Seashore MR, Stricker T, Summar M, Waisbren S, Yudkoff M, Batshaw ML. Establishing a consortium for the study of rare diseases: The Urea Cycle Disorders Consortium. Mol Genet Metab. 2010; 100 Suppl 1:S97-105. PMID: 20188616; PMCID: PMC2858794.
    30. Numata S, Koda Y, Ihara K, Sawada T, Okano Y, Matsuura T, Endo F, Yoo HW, Arranz JA, Rubio V, Wermuth B, Ah Mew N, Tuchman M, Pinner JR, Kirk EP, Yoshino M. Mutant alleles associated with late-onset ornithine transcarbamylase deficiency in male patients have recurrently arisen and have been retained in some populations. J Hum Genet. 2010 Jan; 55(1):18-22. PMID: 19893582.
      View in: PubMed
    31. Ah Mew N, Payan I, Daikhin Y, Nissim I, Nissim I, Tuchman M, Yudkoff M. Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis. Mol Genet Metab. 2009 Dec; 98(4):325-30. PMID: 19660971; PMCID: PMC2784258.
    32. Mitchell S, Ellingson C, Coyne T, Hall L, Neill M, Christian N, Higham C, Dobrowolski SF, Tuchman M, Summar M. Genetic variation in the urea cycle: a model resource for investigating key candidate genes for common diseases. Hum Mutat. 2009 Jan; 30(1):56-60. PMID: 18666241.
      View in: PubMed
    33. Min L, Jin Z, Caldovic L, Morizono H, Allewell NM, Tuchman M, Shi D. Mechanism of allosteric inhibition of N-acetyl-L-glutamate synthase by L-arginine. J Biol Chem. 2009 Feb 20; 284(8):4873-80. PMID: 19095660; PMCID: PMC2643497.
    34. Haskins N, Panglao M, Qu Q, Majumdar H, Cabrera-Luque J, Morizono H, Tuchman M, Caldovic L. Inversion of allosteric effect of arginine on N-acetylglutamate synthase, a molecular marker for evolution of tetrapods. BMC Biochem. 2008 Sep 18; 9:24. PMID: 18801197; PMCID: PMC2566978.
    35. Tuchman M, Caldovic L, Daikhin Y, Horyn O, Nissim I, Nissim I, Korson M, Burton B, Yudkoff M. N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers. Pediatr Res. 2008 Aug; 64(2):213-7. PMID: 18414145; PMCID: PMC2640836.
    36. Tuchman M, Lee B, Lichter-Konecki U, Summar ML, Yudkoff M, Cederbaum SD, Kerr DS, Diaz GA, Seashore MR, Lee HS, McCarter RJ, Krischer JP, Batshaw ML. Cross-sectional multicenter study of patients with urea cycle disorders in the United States. Mol Genet Metab. 2008 Aug; 94(4):397-402. PMID: 18562231; PMCID: PMC2640937.
    37. Klein OD, Kostiner DR, Weisiger K, Moffatt E, Lindeman N, Goodman S, Tuchman M, Packman S. Acute fatal presentation of ornithine transcarbamylase deficiency in a previously healthy male. Hepatol Int. 2008 Sep; 2(3):390-4. PMID: 19669271; PMCID: PMC2716892.
    38. Shi D, Sagar V, Jin Z, Yu X, Caldovic L, Morizono H, Allewell NM, Tuchman M. The crystal structure of N-acetyl-L-glutamate synthase from Neisseria gonorrhoeae provides insights into mechanisms of catalysis and regulation. J Biol Chem. 2008 Mar 14; 283(11):7176-84. PMID: 18184660; PMCID: PMC4099063.
    39. Dobrowolski SF, Ellingson CE, Caldovic L, Tuchman M. Streamlined assessment of gene variants by high resolution melt profiling utilizing the ornithine transcarbamylase gene as a model system. Hum Mutat. 2007 Nov; 28(11):1133-40. PMID: 17565723.
      View in: PubMed
    40. Caldovic L, Morizono H, Tuchman M. Mutations and polymorphisms in the human N-acetylglutamate synthase (NAGS) gene. Hum Mutat. 2007 Aug; 28(8):754-9. PMID: 17421020.
      View in: PubMed
    41. Shi D, Yu X, Cabrera-Luque J, Chen TY, Roth L, Morizono H, Allewell NM, Tuchman M. A single mutation in the active site swaps the substrate specificity of N-acetyl-L-ornithine transcarbamylase and N-succinyl-L-ornithine transcarbamylase. Protein Sci. 2007 Aug; 16(8):1689-99. PMID: 17600144; PMCID: PMC2203365.
    42. Qu Q, Morizono H, Shi D, Tuchman M, Caldovic L. A novel bifunctional N-acetylglutamate synthase-kinase from Xanthomonas campestris that is closely related to mammalian N-acetylglutamate synthase. BMC Biochem. 2007 Apr 10; 8:4. PMID: 17425781; PMCID: PMC1865377.
    43. Shi D, Caldovic L, Jin Z, Yu X, Qu Q, Roth L, Morizono H, Hathout Y, Allewell NM, Tuchman M. Expression, crystallization and preliminary crystallographic studies of a novel bifunctional N-acetylglutamate synthase/kinase from Xanthomonas campestris homologous to vertebrate N-acetylglutamate synthase. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2006 Dec 01; 62(Pt 12):1218-22. PMID: 17142901; PMCID: PMC2225375.
    44. Shi D, Yu X, Roth L, Morizono H, Tuchman M, Allewell NM. Structures of N-acetylornithine transcarbamoylase from Xanthomonas campestris complexed with substrates and substrate analogs imply mechanisms for substrate binding and catalysis. Proteins. 2006 Aug 01; 64(2):532-42. PMID: 16741992.
      View in: PubMed
    45. Yamaguchi S, Brailey LL, Morizono H, Bale AE, Tuchman M. Mutations and polymorphisms in the human ornithine transcarbamylase (OTC) gene. Hum Mutat. 2006 Jul; 27(7):626-32. PMID: 16786505.
      View in: PubMed
    46. Shi D, Yu X, Roth L, Tuchman M, Allewell NM. Structure of a novel N-acetyl-L-citrulline deacetylase from Xanthomonas campestris. Biophys Chem. 2007 Mar; 126(1-3):86-93. PMID: 16750290.
      View in: PubMed
    47. Shi D, Morizono H, Cabrera-Luque J, Yu X, Roth L, Malamy MH, Allewell NM, Tuchman M. Structure and catalytic mechanism of a novel N-succinyl-L-ornithine transcarbamylase in arginine biosynthesis of Bacteroides fragilis. J Biol Chem. 2006 Jul 21; 281(29):20623-31. PMID: 16704984.
      View in: PubMed
    48. Morizono H, Cabrera-Luque J, Shi D, Gallegos R, Yamaguchi S, Yu X, Allewell NM, Malamy MH, Tuchman M. Acetylornithine transcarbamylase: a novel enzyme in arginine biosynthesis. J Bacteriol. 2006 Apr; 188(8):2974-82. PMID: 16585758; PMCID: PMC1446984.
    49. Caldovic L, Lopez GY, Haskins N, Panglao M, Shi D, Morizono H, Tuchman M. Biochemical properties of recombinant human and mouse N-acetylglutamate synthase. Mol Genet Metab. 2006 Mar; 87(3):226-32. PMID: 16321554.
      View in: PubMed
    50. Morizono H, Woolston JE, Colombini M, Tuchman M. The use of yeast mitochondria to study the properties of wild-type and mutant human mitochondrial ornithine transporter. Mol Genet Metab. 2005 Dec; 86(4):431-40. PMID: 16256388.
      View in: PubMed
    51. Shi D, Yu X, Roth L, Morizono H, Hathout Y, Allewell NM, Tuchman M. Expression, purification, crystallization and preliminary X-ray crystallographic studies of a novel acetylcitrulline deacetylase from Xanthomonas campestris. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2005 Jul 01; 61(Pt 7):676-9. PMID: 16511126; PMCID: PMC1952451.
    52. Ensenauer R, Tuchman M, El-Youssef M, Kotagal S, Ishitani MB, Matern D, Babovic-Vuksanovic D. Management and outcome of neonatal-onset ornithine transcarbamylase deficiency following liver transplantation at 60 days of life. Mol Genet Metab. 2005 Apr; 84(4):363-6. PMID: 15781198.
      View in: PubMed
    53. Caldovic L, Morizono H, Panglao MG, Lopez GY, Shi D, Summar ML, Tuchman M. Late onset N-acetylglutamate synthase deficiency caused by hypomorphic alleles. Hum Mutat. 2005 Mar; 25(3):293-8. PMID: 15714518.
      View in: PubMed
    54. Shi D, Morizono H, Yu X, Roth L, Caldovic L, Allewell NM, Malamy MH, Tuchman M. Crystal structure of N-acetylornithine transcarbamylase from Xanthomonas campestris: a novel enzyme in a new arginine biosynthetic pathway found in several eubacteria. J Biol Chem. 2005 Apr 15; 280(15):14366-9. PMID: 15731101.
      View in: PubMed
    55. Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M. Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. J Pediatr. 2004 Oct; 145(4):552-4. PMID: 15480384.
      View in: PubMed
    56. Morizono H, Caldovic L, Shi D, Tuchman M. Mammalian N-acetylglutamate synthase. Mol Genet Metab. 2004 Apr; 81 Suppl 1:S4-11. PMID: 15050968; PMCID: PMC3031861.
    57. MacArthur RB, Altincatal A, Tuchman M. Pharmacokinetics of sodium phenylacetate and sodium benzoate following intravenous administration as both a bolus and continuous infusion to healthy adult volunteers. Mol Genet Metab. 2004 Apr; 81 Suppl 1:S67-73. PMID: 15050977.
      View in: PubMed
    58. Tuchman M. Hyperammonemia: are the burdens too grave? Case study. Ethics Intellect Disabil. 2004; 8(1):1, 3. PMID: 15835081.
      View in: PubMed
    59. Caldovic L, Tuchman M. N-acetylglutamate and its changing role through evolution. Biochem J. 2003 Jun 01; 372(Pt 2):279-90. PMID: 12633501; PMCID: PMC1223426.
    60. Takanashi J, Barkovich AJ, Cheng SF, Weisiger K, Zlatunich CO, Mudge C, Rosenthal P, Tuchman M, Packman S. Brain MR imaging in neonatal hyperammonemic encephalopathy resulting from proximal urea cycle disorders. AJNR Am J Neuroradiol. 2003 Jun-Jul; 24(6):1184-7. PMID: 12812952.
      View in: PubMed
    61. Caldovic L, Morizono H, Panglao MG, Cheng SF, Packman S, Tuchman M. Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia. Hum Genet. 2003 Apr; 112(4):364-8. PMID: 12594532.
      View in: PubMed
    62. Caldovic L, Morizono H, Gracia Panglao M, Gallegos R, Yu X, Shi D, Malamy MH, Allewell NM, Tuchman M. Cloning and expression of the human N-acetylglutamate synthase gene. Biochem Biophys Res Commun. 2002 Dec 13; 299(4):581-6. PMID: 12459178.
      View in: PubMed
    63. Shi D, Gallegos R, DePonte J, Morizono H, Yu X, Allewell NM, Malamy M, Tuchman M. Crystal structure of a transcarbamylase-like protein from the anaerobic bacterium Bacteroides fragilis at 2.0 A resolution. J Mol Biol. 2002 Jul 19; 320(4):899-908. PMID: 12095263.
      View in: PubMed
    64. Caldovic L, Morizono H, Yu X, Thompson M, Shi D, Gallegos R, Allewell NM, Malamy MH, Tuchman M. Identification, cloning and expression of the mouse N-acetylglutamate synthase gene. Biochem J. 2002 Jun 15; 364(Pt 3):825-31. PMID: 12049647; PMCID: PMC1222632.
    65. Woods WG, Gao RN, Shuster JJ, Robison LL, Bernstein M, Weitzman S, Bunin G, Levy I, Brossard J, Dougherty G, Tuchman M, Lemieux B. Screening of infants and mortality due to neuroblastoma. N Engl J Med. 2002 Apr 04; 346(14):1041-6. PMID: 11932470.
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    66. Tuchman M, Jaleel N, Morizono H, Sheehy L, Lynch MG. Mutations and polymorphisms in the human ornithine transcarbamylase gene. Hum Mutat. 2002 Feb; 19(2):93-107. PMID: 11793468.
      View in: PubMed
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