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Jan P Kraus

TitleProfessor
InstitutionUniversity of Colorado Denver - Anschutz Medical Campus
DepartmentSOM-PEDS
Address2RC1N
Research North Tower
12800 E 19th Ave, Bldg. RC1 North Room P18-9103
Aurora CO 80010
Phone303/724-3812

     Bibliographic 
     selected publications
    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. Guenzel AJ, Collard R, Kraus JP, Matern D, Barry MA. Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors. Hum Gene Ther. 2015 Mar; 26(3):153-60.
      View in: PubMed
    2. Thorson MK, Van Wagoner RM, Harper MK, Ireland CM, Majtan T, Kraus JP, Barrios AM. Marine natural products as inhibitors of cystathionine beta-synthase activity. Bioorg Med Chem Lett. 2015 Mar 1; 25(5):1070-2.
      View in: PubMed
    3. Ereño-Orbea J, Majtan T, Oyenarte I, Kraus JP, Martínez-Cruz LA. Structural insight into the molecular mechanism of allosteric activation of human cystathionine ß-synthase by S-adenosylmethionine. Proc Natl Acad Sci U S A. 2014 Sep 16; 111(37):E3845-52.
      View in: PubMed
    4. Majtan T, Pey AL, Fernández R, Fernández JA, Martínez-Cruz LA, Kraus JP. Domain organization, catalysis and regulation of eukaryotic cystathionine beta-synthases. PLoS One. 2014; 9(8):e105290.
      View in: PubMed
    5. Pey AL, Majtan T, Kraus JP. The role of surface electrostatics on the stability, function and regulation of human cystathionine ß-synthase, a complex multidomain and oligomeric protein. Biochim Biophys Acta. 2014 Sep; 1844(9):1453-62.
      View in: PubMed
    6. Ereño-Orbea J, Majtan T, Oyenarte I, Kraus JP, Martínez-Cruz LA. Purification, crystallization and preliminary crystallographic analysis of the catalytic core of cystathionine ß-synthase from Saccharomyces cerevisiae. Acta Crystallogr F Struct Biol Commun. 2014 Mar; 70(Pt 3):320-5.
      View in: PubMed
    7. Damavandi MD, Chan EC, Kraus JP, Ho PC, Kang TS. Development of an UPLC-MS/MS method for assaying the enzymatic activity of propionyl coenzyme-A carboxylase. Bioanalysis. 2014 Feb; 6(3):335-48.
      View in: PubMed
    8. Ereño-Orbea J, Majtan T, Oyenarte I, Kraus JP, Martínez-Cruz LA. Structural basis of regulation and oligomerization of human cystathionine ß-synthase, the central enzyme of transsulfuration. Proc Natl Acad Sci U S A. 2013 Oct 1; 110(40):E3790-9.
      View in: PubMed
    9. Stabler SP, Korson M, Jethva R, Allen RH, Kraus JP, Spector EB, Wagner C, Mudd SH. Metabolic profiling of total homocysteine and related compounds in hyperhomocysteinemia: utility and limitations in diagnosing the cause of puzzling thrombophilia in a family. JIMD Rep. 2013; 11:149-63.
      View in: PubMed
    10. Guenzel AJ, Hofherr SE, Hillestad M, Barry M, Weaver E, Venezia S, Kraus JP, Matern D, Barry MA. Generation of a hypomorphic model of propionic acidemia amenable to gene therapy testing. Mol Ther. 2013 Jul; 21(7):1316-23.
      View in: PubMed
    11. Thorson MK, Majtan T, Kraus JP, Barrios AM. Identification of cystathionine ß-synthase inhibitors using a hydrogen sulfide selective probe. Angew Chem Int Ed Engl. 2013 Apr 22; 52(17):4641-4.
      View in: PubMed
    12. Su Y, Majtan T, Freeman KM, Linck R, Ponter S, Kraus JP, Burstyn JN. Comparative study of enzyme activity and heme reactivity in Drosophila melanogaster and Homo sapiens cystathionine ß-synthases. Biochemistry. 2013 Jan 29; 52(4):741-51.
      View in: PubMed
    13. Grünert SC, Müllerleile S, De Silva L, Barth M, Walter M, Walter K, Meissner T, Lindner M, Ensenauer R, Santer R, Bodamer OA, Baumgartner MR, Brunner-Krainz M, Karall D, Haase C, Knerr I, Marquardt T, Hennermann JB, Steinfeld R, Beblo S, Koch HG, Konstantopoulou V, Scholl-Bürgi S, van Teeffelen-Heithoff A, Suormala T, Sperl W, Kraus JP, Superti-Furga A, Schwab KO, Sass JO. Propionic acidemia: clinical course and outcome in 55 pediatric and adolescent patients. Orphanet J Rare Dis. 2013; 8:6.
      View in: PubMed
    14. Pey AL, Majtan T, Sanchez-Ruiz JM, Kraus JP. Human cystathionine ß-synthase (CBS) contains two classes of binding sites for S-adenosylmethionine (SAM): complex regulation of CBS activity and stability by SAM. Biochem J. 2013 Jan 1; 449(1):109-21.
      View in: PubMed
    15. Oyenarte I, Majtan T, Ereño J, Corral-Rodríguez MA, Kraus JP, Martínez-Cruz LA. Purification, crystallization and preliminary crystallographic analysis of human cystathionine ß-synthase. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2012 Nov 1; 68(Pt 11):1318-22.
      View in: PubMed
    16. Oyenarte I, Majtan T, Ereño J, Corral-Rodríguez MA, Klaudiny J, Majtan J, Kraus JP, Martínez-Cruz LA. Purification, crystallization and preliminary crystallographic analysis of the full-length cystathionine ß-synthase from Apis mellifera. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2012 Nov 1; 68(Pt 11):1323-8.
      View in: PubMed
    17. Smith AT, Su Y, Stevens DJ, Majtan T, Kraus JP, Burstyn JN. Effect of the disease-causing R266K mutation on the heme and PLP environments of human cystathionine ß-synthase. Biochemistry. 2012 Aug 14; 51(32):6360-70.
      View in: PubMed
    18. Hnízda A, Majtan T, Liu L, Pey AL, Carpenter JF, Kodícek M, Kožich V, Kraus JP. Conformational properties of nine purified cystathionine ß-synthase mutants. Biochemistry. 2012 Jun 12; 51(23):4755-63.
      View in: PubMed
    19. Majtan T, Kraus JP. Folding and activity of mutant cystathionine ß-synthase depends on the position and nature of the purification tag: characterization of the R266K CBS mutant. Protein Expr Purif. 2012 Apr; 82(2):317-24.
      View in: PubMed
    20. Régnier V, Billard JM, Gupta S, Potier B, Woerner S, Paly E, Ledru A, David S, Luilier S, Bizot JC, Vacano G, Kraus JP, Patterson D, Kruger WD, Delabar JM, London J. Brain phenotype of transgenic mice overexpressing cystathionine ß-synthase. PLoS One. 2012; 7(1):e29056.
      View in: PubMed
    21. Grünert SC, Müllerleile S, de Silva L, Barth M, Walter M, Walter K, Meissner T, Lindner M, Ensenauer R, Santer R, Bodamer OA, Baumgartner MR, Brunner-Krainz M, Karall D, Haase C, Knerr I, Marquardt T, Hennermann JB, Steinfeld R, Beblo S, Koch HG, Konstantopoulou V, Scholl-Bürgi S, van Teeffelen-Heithoff A, Suormala T, Sperl W, Kraus JP, Superti-Furga A, Schwab KO, Sass JO. Propionic acidemia: neonatal versus selective metabolic screening. J Inherit Metab Dis. 2012 Jan; 35(1):41-9.
      View in: PubMed
    22. Kraus JP, Spector E, Venezia S, Estes P, Chiang PW, Creadon-Swindell G, Müllerleile S, de Silva L, Barth M, Walter M, Walter K, Meissner T, Lindner M, Ensenauer R, Santer R, Bodamer OA, Baumgartner MR, Brunner-Krainz M, Karall D, Haase C, Knerr I, Marquardt T, Hennermann JB, Steinfeld R, Beblo S, Koch HG, Konstantopoulou V, Scholl-Bürgi S, van Teeffelen-Heithoff A, Suormala T, Ugarte M, Sperl W, Superti-Furga A, Schwab KO, Grünert SC, Sass JO. Mutation analysis in 54 propionic acidemia patients. J Inherit Metab Dis. 2012 Jan; 35(1):51-63.
      View in: PubMed
    23. Smith AT, Majtan T, Freeman KM, Su Y, Kraus JP, Burstyn JN. Cobalt cystathionine ß-synthase: a cobalt-substituted heme protein with a unique thiolate ligation motif. Inorg Chem. 2011 May 16; 50(10):4417-27.
      View in: PubMed
    24. Majtan T, Freeman KM, Smith AT, Burstyn JN, Kraus JP. Purification and characterization of cystathionine ß-synthase bearing a cobalt protoporphyrin. Arch Biochem Biophys. 2011 Apr 1; 508(1):25-30.
      View in: PubMed
    25. Majtan T, Frerman FE, Kraus JP. Effect of cobalt on Escherichia coli metabolism and metalloporphyrin formation. Biometals. 2011 Apr; 24(2):335-47.
      View in: PubMed
    26. Hnízda A, Spiwok V, Jurga V, Kozich V, Kodícek M, Kraus JP. Cross-talk between the catalytic core and the regulatory domain in cystathionine ß-synthase: study by differential covalent labeling and computational modeling. Biochemistry. 2010 Dec 14; 49(49):10526-34.
      View in: PubMed
    27. Kozich V, Sokolová J, Klatovská V, Krijt J, Janosík M, Jelínek K, Kraus JP. Cystathionine beta-synthase mutations: effect of mutation topology on folding and activity. Hum Mutat. 2010 Jul; 31(7):809-19.
      View in: PubMed
    28. Maclean KN, Sikora J, Kožich V, Jiang H, Greiner LS, Kraus E, Krijt J, Overdier KH, Collard R, Brodsky GL, Meltesen L, Crnic LS, Allen RH, Stabler SP, Elleder M, Rozen R, Patterson D, Kraus JP. A novel transgenic mouse model of CBS-deficient homocystinuria does not incur hepatic steatosis or fibrosis and exhibits a hypercoagulative phenotype that is ameliorated by betaine treatment. Mol Genet Metab. 2010 Oct-Nov; 101(2-3):153-62.
      View in: PubMed
    29. Maclean KN, Sikora J, Kožich V, Jiang H, Greiner LS, Kraus E, Krijt J, Crnic LS, Allen RH, Stabler SP, Elleder M, Kraus JP. Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment. Mol Genet Metab. 2010 Oct-Nov; 101(2-3):163-71.
      View in: PubMed
    30. Majtan T, Liu L, Carpenter JF, Kraus JP. Rescue of cystathionine beta-synthase (CBS) mutants with chemical chaperones: purification and characterization of eight CBS mutant enzymes. J Biol Chem. 2010 May 21; 285(21):15866-73.
      View in: PubMed
    31. Singh LR, Gupta S, Honig NH, Kraus JP, Kruger WD. Activation of mutant enzyme function in vivo by proteasome inhibitors and treatments that induce Hsp70. PLoS Genet. 2010 Jan; 6(1):e1000807.
      View in: PubMed
    32. Kraus JP. cblC: advances in defining the MMACHC mutation spectrum. Hum Mutat. 2009 Jul; 30(7):v.
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    33. Kraus JP, Hasek J, Kozich V, Collard R, Venezia S, Janosíková B, Wang J, Stabler SP, Allen RH, Jakobs C, Finn CT, Chien YH, Hwu WL, Hegele RA, Mudd SH. Cystathionine gamma-lyase: Clinical, metabolic, genetic, and structural studies. Mol Genet Metab. 2009 Aug; 97(4):250-9.
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    34. Jakubowski H, Perla-Kaján J, Finnell RH, Cabrera RM, Wang H, Gupta S, Kruger WD, Kraus JP, Shih DM. Genetic or nutritional disorders in homocysteine or folate metabolism increase protein N-homocysteinylation in mice. FASEB J. 2009 Jun; 23(6):1721-7.
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    35. Majtan T, Singh LR, Wang L, Kruger WD, Kraus JP. Active cystathionine beta-synthase can be expressed in heme-free systems in the presence of metal-substituted porphyrins or a chemical chaperone. J Biol Chem. 2008 Dec 12; 283(50):34588-95.
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    36. Maillot F, Kraus JP, Lee PJ. Environmental influences on familial discordance of phenotype in people with homocystinuria: a case report. J Med Case Rep. 2008; 2:113.
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    37. Frank N, Kent JO, Meier M, Kraus JP. Purification and characterization of the wild type and truncated human cystathionine beta-synthase enzymes expressed in E. coli. Arch Biochem Biophys. 2008 Feb 1; 470(1):64-72.
      View in: PubMed
    38. Pong WW, Stouracova R, Frank N, Kraus JP, Eldred WD. Comparative localization of cystathionine beta-synthase and cystathionine gamma-lyase in retina: differences between amphibians and mammals. J Comp Neurol. 2007 Nov 10; 505(2):158-65.
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    39. Cherney MM, Pazicni S, Frank N, Marvin KA, Kraus JP, Burstyn JN. Ferrous human cystathionine beta-synthase loses activity during enzyme assay due to a ligand switch process. Biochemistry. 2007 Nov 13; 46(45):13199-210.
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    40. Vyletal P, Sokolová J, Cooper DN, Kraus JP, Krawczak M, Pepe G, Rickards O, Koch HG, Linnebank M, Kluijtmans LA, Blom HJ, Boers GH, Gaustadnes M, Skovby F, Wilcken B, Wilcken DE, Andria G, Sebastio G, Naughten ER, Yap S, Ohura T, Pronicka E, Laszlo A, Kozich V. Diversity of cystathionine beta-synthase haplotypes bearing the most common homocystinuria mutation c.833T>C: a possible role for gene conversion. Hum Mutat. 2007 Mar; 28(3):255-64.
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    41. Frank N, Kery V, Maclean KN, Kraus JP. Solvent-accessible cysteines in human cystathionine beta-synthase: crucial role of cysteine 431 in S-adenosyl-L-methionine binding. Biochemistry. 2006 Sep 12; 45(36):11021-9.
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    42. Bermúdez M, Frank N, Bernal J, Urreizti R, Briceño I, Merinero B, Perez-Cerdá C, Ugarte M, Grinberg D, Balcells S, Kraus JP. High prevalence of CBS p.T191M mutation in homocystinuric patients from Colombia. Hum Mutat. 2006 Mar; 27(3):296.
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    43. Pazicni S, Cherney MM, Lukat-Rodgers GS, Oliveriusová J, Rodgers KR, Kraus JP, Burstyn JN. The heme of cystathionine beta-synthase likely undergoes a thermally induced redox-mediated ligand switch. Biochemistry. 2005 Dec 27; 44(51):16785-95.
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    44. Katsushima F, Oliveriusova J, Sakamoto O, Ohura T, Kondo Y, Iinuma K, Kraus E, Stouracova R, Kraus JP. Expression study of mutant cystathionine beta-synthase found in Japanese patients with homocystinuria. Mol Genet Metab. 2006 Apr; 87(4):323-8.
      View in: PubMed
    45. Jiang H, Rao KS, Yee VC, Kraus JP. Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli. J Biol Chem. 2005 Jul 29; 280(30):27719-27.
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    46. Bar-Or D, Rael LT, Thomas GW, Kraus JP. Inhibitory effect of copper on cystathionine beta-synthase activity: protective effect of an analog of the human albumin N-terminus. Protein Pept Lett. 2005 Apr; 12(3):271-3.
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    47. Bar-Or D, Curtis CG, Sullivan A, Rael LT, Thomas GW, Craun M, Bar-Or R, Maclean KN, Kraus JP. Plasma albumin cysteinylation is regulated by cystathionine beta-synthase. Biochem Biophys Res Commun. 2004 Dec 24; 325(4):1449-53.
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    48. Pazicni S, Lukat-Rodgers GS, Oliveriusová J, Rees KA, Parks RB, Clark RW, Rodgers KR, Kraus JP, Burstyn JN. The redox behavior of the heme in cystathionine beta-synthase is sensitive to pH. Biochemistry. 2004 Nov 23; 43(46):14684-95.
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    49. Linnebank M, Janosik M, Kozich V, Pronicka E, Kubalska J, Sokolova J, Linnebank A, Schmidt E, Leyendecker C, Klockgether T, Kraus JP, Koch HG. The cystathionine beta-synthase (CBS) mutation c.1224-2A>C in Central Europe: Vitamin B6 nonresponsiveness and a common ancestral haplotype. Hum Mutat. 2004 Oct; 24(4):352-3.
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    50. Miles EW, Kraus JP. Cystathionine beta-synthase: structure, function, regulation, and location of homocystinuria-causing mutations. J Biol Chem. 2004 Jul 16; 279(29):29871-4.
      View in: PubMed
    51. Moat SJ, Bao L, Fowler B, Bonham JR, Walter JH, Kraus JP. The molecular basis of cystathionine beta-synthase (CBS) deficiency in UK and US patients with homocystinuria. Hum Mutat. 2004 Feb; 23(2):206.
      View in: PubMed
    52. Maclean KN, Kraus E, Kraus JP. The dominant role of Sp1 in regulating the cystathionine beta-synthase -1a and -1b promoters facilitates potential tissue-specific regulation by Kruppel-like factors. J Biol Chem. 2004 Mar 5; 279(10):8558-66.
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    53. Meier M, Oliveriusova J, Kraus JP, Burkhard P. Structural insights into mutations of cystathionine beta-synthase. Biochim Biophys Acta. 2003 Apr 11; 1647(1-2):206-13.
      View in: PubMed
    54. Orendác M, Zeman J, Stabler SP, Allen RH, Kraus JP, Bodamer O, Stöckler-Ipsiroglu S, Kvasnicka J, Kozich V. Homocystinuria due to cystathionine beta-synthase deficiency: novel biochemical findings and treatment efficacy. J Inherit Metab Dis. 2003; 26(8):761-73.
      View in: PubMed
    55. Oliveriusová J, Kery V, Maclean KN, Kraus JP. Deletion mutagenesis of human cystathionine beta-synthase. Impact on activity, oligomeric status, and S-adenosylmethionine regulation. J Biol Chem. 2002 Dec 13; 277(50):48386-94.
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    56. Ratnam S, Maclean KN, Jacobs RL, Brosnan ME, Kraus JP, Brosnan JT. Hormonal regulation of cystathionine beta-synthase expression in liver. J Biol Chem. 2002 Nov 8; 277(45):42912-8.
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    57. Stabler SP, Steegborn C, Wahl MC, Oliveriusova J, Kraus JP, Allen RH, Wagner C, Mudd SH. Elevated plasma total homocysteine in severe methionine adenosyltransferase I/III deficiency. Metabolism. 2002 Aug; 51(8):981-8.
      View in: PubMed
    58. Levy HL, Vargas JE, Waisbren SE, Kurczynski TW, Roeder ER, Schwartz RS, Rosengren S, Prasad C, Greenberg CR, Gilfix BM, MacGregor D, Shih VE, Bao L, Kraus JP. Reproductive fitness in maternal homocystinuria due to cystathionine beta-synthase deficiency. J Inherit Metab Dis. 2002 Aug; 25(4):299-314.
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    59. Gaustadnes M, Wilcken B, Oliveriusova J, McGill J, Fletcher J, Kraus JP, Wilcken DE. The molecular basis of cystathionine beta-synthase deficiency in Australian patients: genotype-phenotype correlations and response to treatment. Hum Mutat. 2002 Aug; 20(2):117-26.
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    60. Maclean KN, Janosík M, Kraus E, Kozich V, Allen RH, Raab BK, Kraus JP. Cystathionine beta-synthase is coordinately regulated with proliferation through a redox-sensitive mechanism in cultured human cells and Saccharomyces cerevisiae. J Cell Physiol. 2002 Jul; 192(1):81-92.
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    61. Chloupkova M, Maclean KN, Alkhateeb A, Kraus JP. Propionic acidemia: analysis of mutant propionyl-CoA carboxylase enzymes expressed in Escherichia coli. Hum Mutat. 2002 Jun; 19(6):629-40.
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    62. Maclean KN, Gaustadnes M, Oliveriusová J, Janosík M, Kraus E, Kozich V, Kery V, Skovby F, Rüdiger N, Ingerslev J, Stabler SP, Allen RH, Kraus JP. High homocysteine and thrombosis without connective tissue disorders are associated with a novel class of cystathionine beta-synthase (CBS) mutations. Hum Mutat. 2002 Jun; 19(6):641-55.
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    63. Sokolová J, Janosíková B, Terwilliger JD, Freiberger T, Kraus JP, Kozich V. Cystathionine beta-synthase deficiency in Central Europe: discrepancy between biochemical and molecular genetic screening for homocystinuric alleles. Hum Mutat. 2001 Dec; 18(6):548-9.
      View in: PubMed
    64. Janosík M, Kery V, Gaustadnes M, Maclean KN, Kraus JP. Regulation of human cystathionine beta-synthase by S-adenosyl-L-methionine: evidence for two catalytically active conformations involving an autoinhibitory domain in the C-terminal region. Biochemistry. 2001 Sep 4; 40(35):10625-33.
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    65. Meier M, Janosik M, Kery V, Kraus JP, Burkhard P. Structure of human cystathionine beta-synthase: a unique pyridoxal 5'-phosphate-dependent heme protein. EMBO J. 2001 Aug 1; 20(15):3910-6.
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    66. Janosík M, Oliveriusová J, Janosíková B, Sokolová J, Kraus E, Kraus JP, Kozich V. Impaired heme binding and aggregation of mutant cystathionine beta-synthase subunits in homocystinuria. Am J Hum Genet. 2001 Jun; 68(6):1506-13.
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    67. Vadon-Le Goff S, Delaforge M, Boucher JL, Janosik M, Kraus JP, Mansuy D. Coordination chemistry of the heme in cystathionine beta-synthase: formation of iron(II)-isonitrile complexes. Biochem Biophys Res Commun. 2001 May 4; 283(2):487-92.
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    68. Linnebank M, Homberger A, Kraus JP, Harms E, Kozich V, Koch HG. Haplotyping of wild type and I278T alleles of the human cystathionine beta-synthase gene based on a cluster of novel SNPs in IVS12. Hum Mutat. 2001 Apr; 17(4):350-1.
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    69. Janosik M, Meier M, Kery V, Oliveriusova J, Burkhard P, Kraus JP. Crystallization and preliminary X-ray diffraction analysis of the active core of human recombinant cystathionine beta-synthase: an enzyme involved in vascular disease. Acta Crystallogr D Biol Crystallogr. 2001 Feb; 57(Pt 2):289-91.
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    70. Bruno S, Schiaretti F, Burkhard P, Kraus JP, Janosik M, Mozzarelli A. Functional properties of the active core of human cystathionine beta-synthase crystals. J Biol Chem. 2001 Jan 5; 276(1):16-9.
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    71. Chloupková M, Ravn K, Schwartz M, Kraus JP. Changes in the carboxyl terminus of the beta subunit of human propionyl-CoA carboxylase affect the oligomer assembly and catalysis: expression and characterization of seven patient-derived mutant forms of PCC in Escherichia coli. Mol Genet Metab. 2000 Dec; 71(4):623-32.
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    72. Maclean KN, Janosík M, Oliveriusová J, Kery V, Kraus JP. Transsulfuration in Saccharomyces cerevisiae is not dependent on heme: purification and characterization of recombinant yeast cystathionine beta-synthase. J Inorg Biochem. 2000 Aug 31; 81(3):161-71.
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    73. Ravn K, Chloupkova M, Christensen E, Brandt NJ, Simonsen H, Kraus JP, Nielsen IM, Skovby F, Schwartz M. High incidence of propionic acidemia in greenland is due to a prevalent mutation, 1540insCCC, in the gene for the beta-subunit of propionyl CoA carboxylase. Am J Hum Genet. 2000 Jul; 67(1):203-6.
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    74. Kluijtmans LA, Boers GH, Kraus JP, van den Heuvel LP, Cruysberg JR, Trijbels FJ, Blom HJ. The molecular basis of cystathionine beta-synthase deficiency in Dutch patients with homocystinuria: effect of CBS genotype on biochemical and clinical phenotype and on response to treatment. Am J Hum Genet. 1999 Jul; 65(1):59-67.
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    75. Orendác M, Musková B, Richterová E, Zvárová J, Stefek M, Zaykova E, Kraus JP, Stríbrný J, Hyánek J, Kozich V. Is the common 844ins68 polymorphism in the cystathionine beta-synthase gene associated with atherosclerosis? J Inherit Metab Dis. 1999 Jun; 22(5):674-5.
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    76. Kery V, Poneleit L, Meyer JD, Manning MC, Kraus JP. Binding of pyridoxal 5'-phosphate to the heme protein human cystathionine beta-synthase. Biochemistry. 1999 Mar 2; 38(9):2716-24.
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    77. de Franchis R, Kraus E, Kozich V, Sebastio G, Kraus JP. Four novel mutations in the cystathionine beta-synthase gene: effect of a second linked mutation on the severity of the homocystinuric phenotype. Hum Mutat. 1999; 13(6):453-7.
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    78. Kraus JP, Janosík M, Kozich V, Mandell R, Shih V, Sperandeo MP, Sebastio G, de Franchis R, Andria G, Kluijtmans LA, Blom H, Boers GH, Gordon RB, Kamoun P, Tsai MY, Kruger WD, Koch HG, Ohura T, Gaustadnes M. Cystathionine beta-synthase mutations in homocystinuria. Hum Mutat. 1999; 13(5):362-75.
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    79. Kraus JP, Oliveriusová J, Sokolová J, Kraus E, Vlcek C, de Franchis R, Maclean KN, Bao L. The human cystathionine beta-synthase (CBS) gene: complete sequence, alternative splicing, and polymorphisms. Genomics. 1998 Sep 15; 52(3):312-24.
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    80. Gaustadnes M, Kluijtmans LA, Jensen OK, Rasmussen K, Heil SG, Kraus JP, Blom HJ, Ingerslev J, Rüdiger N. Detection of a novel deletion in the cystathionine beta-synthase (CBS) gene using an improved genomic DNA based method. FEBS Lett. 1998 Jul 17; 431(2):175-9.
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    81. Kery V, Poneleit L, Kraus JP. Trypsin cleavage of human cystathionine beta-synthase into an evolutionarily conserved active core: structural and functional consequences. Arch Biochem Biophys. 1998 Jul 15; 355(2):222-32.
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    82. Kraus JP. Biochemistry and molecular genetics of cystathionine beta-synthase deficiency. Eur J Pediatr. 1998 Apr; 157 Suppl 2:S50-3.
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    83. Bao L, Vlcek C, Paces V, Kraus JP. Identification and tissue distribution of human cystathionine beta-synthase mRNA isoforms. Arch Biochem Biophys. 1998 Feb 1; 350(1):95-103.
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    84. Gordon RB, Cox AJ, Dawson PA, Emmerson BT, Kraus JP, Dudman NP. Mutational analysis of the cystathionine beta-synthase gene: a splicing mutation, two missense mutations and an insertion in patients with homocystinuria. Mutations in brief no. 120. Online. Hum Mutat. 1998; 11(4):332.
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    85. Dong A, Kery V, Matsuura J, Manning MC, Kraus JP, Carpenter JF. Secondary structure of recombinant human cystathionine beta-synthase in aqueous solution: effect of ligand binding and proteolytic truncation. Arch Biochem Biophys. 1997 Aug 1; 344(1):125-32.
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    86. Kozich V, Janosík M, Sokolová J, Oliveriusová J, Orendác M, Kraus JP, Elleder D. Analysis of CBS alleles in Czech and Slovak patients with homocystinuria: report on three novel mutations E176K, W409X and 1223 + 37 del99. J Inherit Metab Dis. 1997 Jul; 20(3):363-6.
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    87. Dawson PA, Cox AJ, Emmerson BT, Dudman NP, Kraus JP, Gordon RB. Characterisation of five missense mutations in the cystathionine beta-synthase gene from three patients with B6-nonresponsive homocystinuria. Eur J Hum Genet. 1997 Jan-Feb; 5(1):15-21.
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    88. Roper MD, Straubhaar JR, Kraus E, Sokolová J, Hrebícek M, Kraus JP. Comparison of the 5' end of the rat and mouse cystathionine beta-synthase genes. Mamm Genome. 1996 Oct; 7(10):754-7.
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    89. Kluijtmans LA, Boers GH, Stevens EM, Renier WO, Kraus JP, Trijbels FJ, van den Heuvel LP, Blom HJ. Defective cystathionine beta-synthase regulation by S-adenosylmethionine in a partially pyridoxine responsive homocystinuria patient. J Clin Invest. 1996 Jul 15; 98(2):285-9.
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    90. Dawson PA, Cochran DA, Emmerson BT, Kraus JP, Dudman NP, Gordon RB. Variable hyperhomocysteinaemia phenotype in heterozygotes for the Gly307Ser mutation in cystathionine beta-synthase. Aust N Z J Med. 1996 Apr; 26(2):180-5.
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    91. Kelson TL, Ohura T, Kraus JP. Chaperonin-mediated assembly of wild-type and mutant subunits of human propionyl-CoA carboxylase expressed in Escherichia coli. Hum Mol Genet. 1996 Mar; 5(3):331-7.
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    92. Shih VE, Fringer JM, Mandell R, Kraus JP, Berry GT, Heidenreich RA, Korson MS, Levy HL, Ramesh V. A missense mutation (I278T) in the cystathionine beta-synthase gene prevalent in pyridoxine-responsive homocystinuria and associated with mild clinical phenotype. Am J Hum Genet. 1995 Jul; 57(1):34-9.
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    93. Sebastio G, Sperandeo MP, Panico M, de Franchis R, Kraus JP, Andria G. The molecular basis of homocystinuria due to cystathionine beta-synthase deficiency in Italian families, and report of four novel mutations. Am J Hum Genet. 1995 Jun; 56(6):1324-33.
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    94. Kozich V, Kraus E, de Franchis R, Fowler B, Boers GH, Graham I, Kraus JP. Hyperhomocysteinemia in premature arterial disease: examination of cystathionine beta-synthase alleles at the molecular level. Hum Mol Genet. 1995 Apr; 4(4):623-9.
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    95. Kery V, Elleder D, Kraus JP. Delta-aminolevulinate increases heme saturation and yield of human cystathionine beta-synthase expressed in Escherichia coli. Arch Biochem Biophys. 1995 Jan 10; 316(1):24-9.
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    96. Sperandeo MP, Panico M, Pepe A, Candito M, de Franchis R, Kraus JP, Andria G, Sebastio G. Molecular analysis of patients affected by homocystinuria due to cystathionine beta-synthase deficiency: report of a new mutation in exon 8 and a deletion in intron 11. J Inherit Metab Dis. 1995; 18(2):211-4.
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    97. Gallagher PM, Ward P, Tan S, Naughten E, Kraus JP, Sellar GC, McConnell DJ, Graham I, Whitehead AS. High frequency (71%) of cystathionine beta-synthase mutation G307S in Irish homocystinuria patients. Hum Mutat. 1995; 6(2):177-80.
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    98. Kery V, Bukovska G, Kraus JP. Transsulfuration depends on heme in addition to pyridoxal 5'-phosphate. Cystathionine beta-synthase is a heme protein. J Biol Chem. 1994 Oct 14; 269(41):25283-8.
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    99. Marble M, Geraghty MT, de Franchis R, Kraus JP, Valle D. Characterization of a cystathionine beta-synthase allele with three mutations in cis in a patient with B6 nonresponsive homocystinuria. Hum Mol Genet. 1994 Oct; 3(10):1883-6.
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    100. Bukovska G, Kery V, Kraus JP. Expression of human cystathionine beta-synthase in Escherichia coli: purification and characterization. Protein Expr Purif. 1994 Oct; 5(5):442-8.
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    101. de Franchis R, Kozich V, McInnes RR, Kraus JP. Identical genotypes in siblings with different homocystinuric phenotypes: identification of three mutations in cystathionine beta-synthase using an improved bacterial expression system. Hum Mol Genet. 1994 Jul; 3(7):1103-8.
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    102. Kraus JP. Komrower Lecture. Molecular basis of phenotype expression in homocystinuria. J Inherit Metab Dis. 1994; 17(4):383-90.
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    103. Hu FL, Gu Z, Kozich V, Kraus JP, Ramesh V, Shih VE. Molecular basis of cystathionine beta-synthase deficiency in pyridoxine responsive and nonresponsive homocystinuria. Hum Mol Genet. 1993 Nov; 2(11):1857-60.
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    104. Kraus JP, Le K, Swaroop M, Ohura T, Tahara T, Rosenberg LE, Roper MD, Kozich V. Human cystathionine beta-synthase cDNA: sequence, alternative splicing and expression in cultured cells. Hum Mol Genet. 1993 Oct; 2(10):1633-8.
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    105. Kozich V, de Franchis R, Kraus JP. Molecular defect in a patient with pyridoxine-responsive homocystinuria. Hum Mol Genet. 1993 Jun; 2(6):815-6.
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    106. Avramopoulos D, Cox T, Kraus JP, Chakravarti A, Antonarakis SE. Linkage mapping of the cystathionine beta-synthase (CBS) gene on human chromosome 21 using a DNA polymorphism in the 3' untranslated region. Hum Genet. 1993 Jan; 90(5):566-8.
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    107. Tahara T, Kraus JP, Ohura T, Rosenberg LE, Fenton WA. Three independent mutations in the same exon of the PCCB gene: differences between Caucasian and Japanese propionic acidaemia. J Inherit Metab Dis. 1993; 16(2):353-60.
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    108. Kraus JP, Tahara T. Direct DNA sequencing of polymerase chain reaction-amplified genomic DNA by Maxam-Gilbert method. Methods Enzymol. 1993; 218:227-33.
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    109. Roper MD, Kraus JP. Rat cystathionine beta-synthase: expression of four alternatively spliced isoforms in transfected cultured cells. Arch Biochem Biophys. 1992 Nov 1; 298(2):514-21.
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    110. Swaroop M, Bradley K, Ohura T, Tahara T, Roper MD, Rosenberg LE, Kraus JP. Rat cystathionine beta-synthase. Gene organization and alternative splicing. J Biol Chem. 1992 Jun 5; 267(16):11455-61.
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    111. Kozich V, Kraus JP. Screening for mutations by expressing patient cDNA segments in E. coli: homocystinuria due to cystathionine beta-synthase deficiency. Hum Mutat. 1992; 1(2):113-23.
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    112. Kraus JP, Kozich V. Pyridoxine responsive and unresponsive homocystinuria. J Nutr Sci Vitaminol (Tokyo). 1992; Spec No:589-92.
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    113. Wagstaff J, Korson M, Kraus JP, Levy HL. Severe folate deficiency and pancytopenia in a nutritionally deprived infant and homocystinuria caused by cystathionine beta-synthase deficiency. J Pediatr. 1991 Apr; 118(4 Pt 1):569-72.
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    114. Nadeau JH, Herrmann B, Bucan M, Burkart D, Crosby JL, Erhart MA, Kosowsky M, Kraus JP, Michiels F, Schnattinger A, et al. Genetic maps of mouse chromosome 17 including 12 new anonymous DNA loci and 25 anchor loci. Genomics. 1991 Jan; 9(1):78-89.
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    115. Threadgill DS, Kraus JP, Krawetz SA, Womack JE. Evidence for the evolutionary origin of human chromosome 21 from comparative gene mapping in the cow and mouse. Proc Natl Acad Sci U S A. 1991 Jan 1; 88(1):154-8.
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    116. Tahara T, Eto Y, Kraus JP, Rosenberg LE. [Molecular basis of organic acidemia--propionic acidemia]. Hum Cell. 1990 Dec; 3(4):311-7.
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    117. Tahara T, Kraus JP, Rosenberg LE. Direct DNA sequencing of PCR amplified genomic DNA by the Maxam-Gilbert method. Biotechniques. 1990 Apr; 8(4):366-8.
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    118. Tahara T, Kraus JP, Rosenberg LE. An unusual insertion/deletion in the gene encoding the beta-subunit of propionyl-CoA carboxylase is a frequent mutation in Caucasian propionic acidemia. Proc Natl Acad Sci U S A. 1990 Feb; 87(4):1372-6.
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    119. Kraus JP. Molecular analysis of cystathionine beta-synthase--a gene on chromosome 21. Prog Clin Biol Res. 1990; 360:201-14.
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    120. Locker J, Gill TJ, Kraus JP, Ohura T, Swarop M, Rivière M, Islam MQ, Levan G, Szpirer J, Szpirer C. The rat MHC and cystathionine beta-synthase gene are syntenic on chromosome 20. Immunogenetics. 1990; 31(4):271-4.
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    121. Ohura T, Kraus JP, Rosenberg LE. Unequal synthesis and differential degradation of propionyl CoA carboxylase subunits in cells from normal and propionic acidemia patients. Am J Hum Genet. 1989 Jul; 45(1):33-40.
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    122. Szpirer C, Kraus JP, Rivière M, Swaroop M, Ohura T, Matsubara Y, Szpirer J, Islam MQ, Levan G. Assignment of the rat genes coding for medium-chain acyl-CoA dehydrogenase, isovaleryl-CoA dehydrogenase, and the beta subunit of propionyl-CoA carboxylase to chromosomes 2, 3, and 8, respectively. Cytogenet Cell Genet. 1989; 50(1):23-6.
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    123. Kraus JP, Novotný J, Kalousek F, Swaroop M, Rosenberg LE. Different structures in the amino-terminal domain of the ornithine transcarbamylase leader peptide are involved in mitochondrial import and carboxyl-terminal cleavage. Proc Natl Acad Sci U S A. 1988 Dec; 85(23):8905-9.
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    124. Münke M, Kraus JP, Ohura T, Francke U. The gene for cystathionine beta-synthase (CBS) maps to the subtelomeric region on human chromosome 21q and to proximal mouse chromosome 17. Am J Hum Genet. 1988 Apr; 42(4):550-9.
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    125. Sztul ES, Hendrick JP, Kraus JP, Wall D, Kalousek F, Rosenberg LE. Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide. J Cell Biol. 1987 Dec; 105(6 Pt 1):2631-9.
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    126. Kraus JP, Matsubara Y, Barton D, Yang-Feng TL, Glassberg R, Ito M, Ikeda Y, Mole J, Francke U, Tanaka K. Isolation of cDNA clones coding for rat isovaleryl-CoA dehydrogenase and assignment of the gene to human chromosome 15. Genomics. 1987 Nov; 1(3):264-9.
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    127. Matsubara Y, Kraus JP, Ozasa H, Glassberg R, Finocchiaro G, Ikeda Y, Mole J, Rosenberg LE, Tanaka K. Molecular cloning and nucleotide sequence of cDNA encoding the entire precursor of rat liver medium chain acyl coenzyme A dehydrogenase. J Biol Chem. 1987 Jul 25; 262(21):10104-8.
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    128. Fenton WA, Hack AM, Kraus JP, Rosenberg LE. Immunochemical studies of fibroblasts from patients with methylmalonyl-CoA mutase apoenzyme deficiency: detection of a mutation interfering with mitochondrial import. Proc Natl Acad Sci U S A. 1987 Mar; 84(5):1421-4.
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    129. Kraus JP. Cystathionine beta-synthase (human). Methods Enzymol. 1987; 143:388-94.
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    130. Kraus JP, Firgaira F, Novotný J, Kalousek F, Williams KR, Williamson C, Ohura T, Rosenberg LE. Coding sequence of the precursor of the beta subunit of rat propionyl-CoA carboxylase. Proc Natl Acad Sci U S A. 1986 Nov; 83(21):8049-53.
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    131. Matsubara Y, Kraus JP, Yang-Feng TL, Francke U, Rosenberg LE, Tanaka K. Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human chromosome 1. Proc Natl Acad Sci U S A. 1986 Sep; 83(17):6543-7.
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    132. Kraus JP, Williamson CL, Firgaira FA, Yang-Feng TL, Münke M, Francke U, Rosenberg LE. Cloning and screening with nanogram amounts of immunopurified mRNAs: cDNA cloning and chromosomal mapping of cystathionine beta-synthase and the beta subunit of propionyl-CoA carboxylase. Proc Natl Acad Sci U S A. 1986 Apr; 83(7):2047-51.
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    133. Rosenberg LE, Fenton WA, Horwich AL, Kalousek F, Kraus JP. Targeting of nuclear-encoded proteins to the mitochondrial matrix: implications for human genetic defects. Ann N Y Acad Sci. 1986; 488:99-108.
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    134. Kraus JP, Hodges PE, Williamson CL, Horwich AL, Kalousek F, Williams KR, Rosenberg LE. A cDNA clone for the precursor of rat mitochondrial ornithine transcarbamylase: comparison of rat and human leader sequences and conservation of catalytic sites. Nucleic Acids Res. 1985 Feb 11; 13(3):943-52.
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    135. Firgaira FA, Hendrick JP, Kalousek F, Kraus JP, Rosenberg LE. RNA required for import of precursor proteins into mitochondria. Science. 1984 Dec 14; 226(4680):1319-22.
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    136. Horwich AL, Fenton WA, Williams KR, Kalousek F, Kraus JP, Doolittle RF, Konigsberg W, Rosenberg LE. Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase. Science. 1984 Jun 8; 224(4653):1068-74.
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    137. Skovby F, Kraus JP, Rosenberg LE. Homocystinuria: biogenesis of cystathionine beta-synthase subunits in cultured fibroblasts and in an in vitro translation system programmed with fibroblast messenger RNA. Am J Hum Genet. 1984 Mar; 36(2):452-9.
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    138. Skovby F, Kraus JP, Rosenberg LE. Biosynthesis of human cystathionine beta-synthase in cultured fibroblasts. J Biol Chem. 1984 Jan 10; 259(1):583-7.
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    139. Skovby F, Kraus JP, Rosenberg LE. Biosynthesis and proteolytic activation of cystathionine beta-synthase in rat liver. J Biol Chem. 1984 Jan 10; 259(1):588-93.
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    140. Horwich AL, Kraus JP, Williams K, Kalousek F, Konigsberg W, Rosenberg LE. Molecular cloning of the cDNA coding for rat ornithine transcarbamoylase. Proc Natl Acad Sci U S A. 1983 Jul; 80(14):4258-62.
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    141. Kraus JP, Kalousek F, Rosenberg LE. Biosynthesis and mitochondrial processing of the beta subunit of propionyl coenzyme A carboxylase from rat liver. J Biol Chem. 1983 Jun 25; 258(12):7245-8.
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    142. Kraus JP, Rosenberg LE. Cystathionine beta-synthase from human liver: improved purification scheme and additional characterization of the enzyme in crude and pure form. Arch Biochem Biophys. 1983 Apr 1; 222(1):44-52.
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    143. Kraus JP, Rosenberg LE. Purification of low-abundance messenger RNAs from rat liver by polysome immunoadsorption. Proc Natl Acad Sci U S A. 1982 Jul; 79(13):4015-9.
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    144. Rosenberg LE, Desnick RJ, Bach G, van Diggelen OP, d'Azzo A, Mueller OT, Wolf B, Kraus JP, Velazquez A, Berman M, Chemke J. Workshop on inborn errors of metabolism. Prog Clin Biol Res. 1982; 103 Pt B:223-9.
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    145. Kraus JP, Conboy JG, Rosenberg LE. Pre-ornithine transcarbamylase. Properties of the cytoplasmic precursor of a mitochondrial matrix enzyme. J Biol Chem. 1981 Nov 10; 256(21):10739-42.
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    146. Lipson MH, Kraus JP, Solomon LR, Rosenberg LE. Depletion of cultured human fibroblasts of pyridoxal 5'-phosphate: effect on activities of aspartate aminotransferase, alanine aminotransferase, and cystathionine beta-synthase. Arch Biochem Biophys. 1980 Oct 15; 204(2):486-93.
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