Jan Kraus, PhD
Professor, Pediatrics-Clinical Genetics and Metabolism

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Graduate School :
  • PhD, University of Basel (1972)
Languages: English
Department: Pediatrics-Clinical Genetics and Metabolism

Research Interests

My laboratory studies the molecular basis of two inherited metabolic disorders associated with deficiency of cystathionine ß-synthase (CBS) and propionyl CoA carboxylase (PCC).

Publications

  • Majtan T, Bublil EM, Park I, Arning E, Bottiglieri T, Glavin F, Kraus JP. Pharmacokinetics and pharmacodynamics of PEGylated truncated human cystathionine beta-synthase for treatment of homocystinuria. Life Sci. 2018 May 1;200:15-25. PubMed PMID: 29526799
  • Majtan T, Jones W Jr, Krijt J, Park I, Kruger WD, Kožich V, Bassnett S, Bublil EM, Kraus JP. Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria. Mol Ther. 2018 Mar 7;26(3):834-844. PubMed PMID: 29398487
  • Collard R, Majtan T, Park I, Kraus JP. Import of TAT-Conjugated Propionyl Coenzyme A Carboxylase Using Models of Propionic Acidemia. Mol Cell Biol. 2018 Mar 15;38(6). PubMed PMID: 29378828
  • Giménez-Mascarell P, Majtan T, Oyenarte I, Ereño-Orbea J, Majtan J, Klaudiny J, Kraus JP, Martínez-Cruz LA. Crystal structure of cystathionine ß-synthase from honeybee Apis mellifera. J Struct Biol. 2018 Apr;202(1):82-93. PubMed PMID: 29275181
  • Majtan T, Pey AL, Gimenez-Mascarell P, Martínez-Cruz LA, Szabo C, Kožich V, Kraus JP. Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria. Handb Exp Pharmacol. 2017 Nov 10. [Epub ahead of print] PubMed PMID: 29119254
  • Majtan T, Park I, Bublil EM, Kraus JP. Enzyme replacement therapy prevents loss of bone and fat mass in murine homocystinuria. Hum Mutat. 2017 Oct 16. [Epub ahead of print] PubMed PMID: 29044829
  • Majtan T, Krijt J, Sokolova J, Krizkova M, Ralat M, Kent J, Gregory Iii JF, Kozich V, Kraus J. Biogenesis of Hydrogen Sulfide and Thioethers by Cystathionine Beta-Synthase. Antioxid Redox Signal. 2017 Sep 6. [Epub ahead of print] PubMed PMID: 28874062
  • Pey AL, Martínez-Cruz LA, Kraus JP, Majtan T. Oligomeric status of human cystathionine beta-synthase modulates AdoMet binding. FEBS Lett. 2016 Nov 14. [Epub ahead of print] PubMed PMID: 27861796
  • Bublil EM, Majtan T, Park I, Carrillo RS, Hulková H, Krijt J, Kožich V, Kraus JP. Enzyme replacement with PEGylated cystathionine ß-synthase ameliorates homocystinuria in murine model. J Clin Invest. 2016 Jun 1;126(6):2372-84. PubMed PMID: 27183385
  • Majtan T, Pey AL, Ereño-Orbea J, Martínez-Cruz LA, Kraus JP. Targeting Cystathionine Beta-Synthase Misfolding in Homocystinuria by Small Ligands: State of the Art and Future Directions. Curr Drug Targets. 2016;17(13):1455-70. PubMed PMID: 26931358
  • Majtan T, Pey AL, Kraus JP. Kinetic stability of cystathionine beta-synthase can be modulated by structural analogs of S-adenosylmethionine: Potential approach to pharmacological chaperone therapy for homocystinuria. Biochimie. 2016 Jul;126:6-13. PubMed PMID: 26805382
  • Kožich V, Krijt J, Sokolová J, Melenovská P, Ješina P, Vozdek R, Majtán T, Kraus JP. Thioethers as markers of hydrogen sulfide production in homocystinurias. Biochimie. 2016 Jul;126:14-20. PubMed PMID: 26791043
  • 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):1064-6. PubMed PMID: 25666819
  • 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. PubMed PMID: 25654275
  • 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. PubMed PMID: 24471954
  • 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. PubMed PMID: 25197074
  • 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. PubMed PMID: 25122507
  • 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. PubMed PMID: 24780582
  • 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. PubMed PMID: 24598918
  • 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. PubMed PMID: 24471954
  • 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. PubMed PMID: 24043838
  • 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. PubMed PMID: 23733603
  • 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. PubMed PMID: 23648696
  • 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. PubMed PMID: 23512751
  • 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 Jan 10;8:6. PubMed PMID: 23305374
  • Su Y, Majtan T, Freeman KM, Linck RC, Ponter S, Kraus JP, Burstyn JN. Comparative study of enzyme activity and heme reactivity in Drosophila melanogaster and Homo sapiens cystathionine beta-synthases. Biochemistry. 2012 Sep 24. [Epub ahead of print] PubMed PMID: 23002992
  • 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. PubMed PMID: 22985361
  • 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. PubMed PMID: 23143241
  • 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. PubMed PMID: 23143240
  • 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. PubMed PMID: 22738154
  • Hni´zda A, Majtan T, Liu L, Pey AL, Carpenter JF, Kodi´cek M, Kožich V, Kraus JP. Correction to Conformational Properties of Nine Purified Cystathionine ß-Synthase Mutants. Biochemistry. 2012 Jul 10;51(27):5540. PubMed PMID: 22734603
  • 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. PubMed PMID: 22612060
  • 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. PubMed PMID: 22333527
  • 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. PubMed PMID: 22253703
  • 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. PubMed PMID: 22134541
  • 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. PubMed PMID: 22033733
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Practice Locations

University of Colorado Hospital (UCH)
12605 E. 16th Ave
Aurora, CO 80045
720-848-0000

Hospital Affiliation
  • Children's Hospital Colorado

Specialty Information

Specialties
  • Psychology
Conditions & Treatments
  • Genetic Conditions and Testing

General Information

Graduate Schools:
  • PhD, University of Basel (1972)
Languages: English
Department: Pediatrics-Clinical Genetics and Metabolism
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