JAN KRAUS

Summary

Affiliation: University of Colorado Health Sciences Center
Country: USA

Publications

  1. ncbi request reprint The human cystathionine beta-synthase (CBS) gene: complete sequence, alternative splicing, and polymorphisms
    J P Kraus
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    Genomics 52:312-24. 1998
  2. ncbi request reprint Cystathionine beta-synthase mutations in homocystinuria
    J P Kraus
    Department of Pediatrics, University of Colorado School of Medicine, Denver 80262, USA
    Hum Mutat 13:362-75. 1999
  3. ncbi request reprint Solvent-accessible cysteines in human cystathionine beta-synthase: crucial role of cysteine 431 in S-adenosyl-L-methionine binding
    Nina Frank
    Department of Pediatrics, University of Colorado School of Medicine at Fitzsimons, 12800 East 19th Avenue, Mail Stop 8313, P O Box 6511, Aurora, Colorado 80045 0511, USA
    Biochemistry 45:11021-9. 2006
  4. ncbi request reprint Ferrous human cystathionine beta-synthase loses activity during enzyme assay due to a ligand switch process
    Melisa M Cherney
    Department of Chemistry, University of Wisconsin, Madison, Wisconsin 53706, USA
    Biochemistry 46:13199-210. 2007
  5. ncbi request reprint The dominant role of Sp1 in regulating the cystathionine beta-synthase -1a and -1b promoters facilitates potential tissue-specific regulation by Kruppel-like factors
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    J Biol Chem 279:8558-66. 2004
  6. ncbi request reprint High homocysteine and thrombosis without connective tissue disorders are associated with a novel class of cystathionine beta-synthase (CBS) mutations
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado, USA
    Hum Mutat 19:641-55. 2002
  7. pmc Purification and characterization of the wild type and truncated human cystathionine beta-synthase enzymes expressed in E. coli
    Nina Frank
    Department of Pediatrics, University of Colorado School of Medicine, UCHSC, RC1 North, Rm 4128, 12800, Mail Stop 8313, P O Box 6511, Aurora, CO 80045 0511, USA
    Arch Biochem Biophys 470:64-72. 2008
  8. ncbi request reprint Deletion mutagenesis of human cystathionine beta-synthase. Impact on activity, oligomeric status, and S-adenosylmethionine regulation
    Jana Oliveriusova
    Department of Pediatrics, University of Colorado School of Medicine, Denver 80262, USA
    J Biol Chem 277:48386-94. 2002
  9. pmc Active cystathionine beta-synthase can be expressed in heme-free systems in the presence of metal-substituted porphyrins or a chemical chaperone
    Tomas Majtan
    Department of Pediatrics, University of Colorado, Denver, Aurora, Colorado 80045, USA
    J Biol Chem 283:34588-95. 2008
  10. ncbi request reprint Cystathionine beta-synthase is coordinately regulated with proliferation through a redox-sensitive mechanism in cultured human cells and Saccharomyces cerevisiae
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    J Cell Physiol 192:81-92. 2002

Research Grants

  1. Homocysteine & Heme: A Role in Thrombosis?
    JAN KRAUS; Fiscal Year: 2009

Collaborators

  • Kenneth N Maclean
  • R B Gordon
  • M Y Tsai
  • G H Boers
  • M Linnebank
  • D N Cooper
  • Warren Kruger
  • B Wilcken
  • D Bar-Or
  • Michael Krawczak
  • Jana Oliveriusova
  • Nina Frank
  • Samuel Pazicni
  • Melisa M Cherney
  • Judith N Burstyn
  • Markus Meier
  • Mette Gaustadnes
  • Tomas Majtan
  • Petr Vyletal
  • Toshihiro Ohura
  • Fumio Katsushima
  • Marta Bermudez
  • Hua Jiang
  • Gudrun S Lukat-Rodgers
  • Kenton R Rodgers
  • Edith Wilson Miles
  • Stuart J Moat
  • Vladimir Kery
  • Shobhitha Ratnam
  • Sally P Stabler
  • Liqun Wang
  • Laishram R Singh
  • Jana O Kent
  • Katherine A Marvin
  • Aranka Laszlo
  • Olga Rickards
  • David E L Wilcken
  • Jitka Sokolova
  • Gianfranco Sebastio
  • Flemming Skovby
  • Viktor Kozich
  • Leo A J Kluijtmans
  • Henk J Blom
  • Guglielmina Pepe
  • Hans G Koch
  • Ewa Pronicka
  • Sufin Yap
  • Eileen R Naughten
  • Generoso Andria
  • Roser Urreizti
  • Celia Perez-Cerda
  • Renata Stouracova
  • Kazuie Iinuma
  • Eva Kraus
  • Jaime Bernal
  • Magdalena Ugarte
  • Begoña Merinero
  • Osamu Sakamoto
  • Yoshiaki Kondo
  • Susana Balcells
  • Ignacio Briceno
  • Daniel Grinberg
  • Vivien C Yee
  • K Sudhindra Rao
  • Katherine A Rees
  • Liming Bao
  • Brian Fowler
  • Robert W Clark
  • Ryan B Parks
  • John H Walter
  • James R Bonham
  • Peter Burkhard
  • Robert H Allen
  • Conrad Wagner
  • Clemens Steegborn
  • Markus C Wahl
  • Rene L Jacobs
  • Margaret E Brosnan
  • David E Wilcken
  • John T Brosnan
  • Jim McGill
  • S Harvey Mudd
  • Janice Fletcher

Detail Information

Publications24

  1. ncbi request reprint The human cystathionine beta-synthase (CBS) gene: complete sequence, alternative splicing, and polymorphisms
    J P Kraus
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    Genomics 52:312-24. 1998
    ..Additionally, we report on a number of DNA sequence repeats that are polymorphic in North American and European Caucasians...
  2. ncbi request reprint Cystathionine beta-synthase mutations in homocystinuria
    J P Kraus
    Department of Pediatrics, University of Colorado School of Medicine, Denver 80262, USA
    Hum Mutat 13:362-75. 1999
    ..Mutations due to deaminations of methylcytosines represent 53% of all point substitutions in the coding region of the CBS gene...
  3. ncbi request reprint Solvent-accessible cysteines in human cystathionine beta-synthase: crucial role of cysteine 431 in S-adenosyl-L-methionine binding
    Nina Frank
    Department of Pediatrics, University of Colorado School of Medicine at Fitzsimons, 12800 East 19th Avenue, Mail Stop 8313, P O Box 6511, Aurora, Colorado 80045 0511, USA
    Biochemistry 45:11021-9. 2006
    ....
  4. ncbi request reprint Ferrous human cystathionine beta-synthase loses activity during enzyme assay due to a ligand switch process
    Melisa M Cherney
    Department of Chemistry, University of Wisconsin, Madison, Wisconsin 53706, USA
    Biochemistry 46:13199-210. 2007
    ..The slow and irreversible loss of FeII CBS enzyme activity in vitro resembles protein denaturation, and suggests that a simple regulatory mechanism based on the heme redox state is unlikely...
  5. ncbi request reprint The dominant role of Sp1 in regulating the cystathionine beta-synthase -1a and -1b promoters facilitates potential tissue-specific regulation by Kruppel-like factors
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    J Biol Chem 279:8558-66. 2004
    ....
  6. ncbi request reprint High homocysteine and thrombosis without connective tissue disorders are associated with a novel class of cystathionine beta-synthase (CBS) mutations
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado, USA
    Hum Mutat 19:641-55. 2002
    ....
  7. pmc Purification and characterization of the wild type and truncated human cystathionine beta-synthase enzymes expressed in E. coli
    Nina Frank
    Department of Pediatrics, University of Colorado School of Medicine, UCHSC, RC1 North, Rm 4128, 12800, Mail Stop 8313, P O Box 6511, Aurora, CO 80045 0511, USA
    Arch Biochem Biophys 470:64-72. 2008
    ..The activity of the enzyme was unaffected by the redox status of the heme iron. Finally, we show that CBS is stimulated by S-adenosyl- l-methionine but not its analogs...
  8. ncbi request reprint Deletion mutagenesis of human cystathionine beta-synthase. Impact on activity, oligomeric status, and S-adenosylmethionine regulation
    Jana Oliveriusova
    Department of Pediatrics, University of Colorado School of Medicine, Denver 80262, USA
    J Biol Chem 277:48386-94. 2002
    ....
  9. pmc Active cystathionine beta-synthase can be expressed in heme-free systems in the presence of metal-substituted porphyrins or a chemical chaperone
    Tomas Majtan
    Department of Pediatrics, University of Colorado, Denver, Aurora, Colorado 80045, USA
    J Biol Chem 283:34588-95. 2008
    ....
  10. ncbi request reprint Cystathionine beta-synthase is coordinately regulated with proliferation through a redox-sensitive mechanism in cultured human cells and Saccharomyces cerevisiae
    Kenneth N Maclean
    Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado 80262, USA
    J Cell Physiol 192:81-92. 2002
    ..Our data indicates that screening compounds for the ability to affect transsulfuration in cultured cell models must take proliferation status into account to avoid masking regulatory interactions that may be of significance in vivo...
  11. ncbi request reprint Elevated plasma total homocysteine in severe methionine adenosyltransferase I/III deficiency
    Sally P Stabler
    Department of Medicine, University of Colorado Health Sciences Center, Denver, CO 80262, USA
    Metabolism 51:981-8. 2002
    ....
  12. ncbi request reprint Structural insights into mutations of cystathionine beta-synthase
    Markus Meier
    M E Müller Institute for Structural Biology, Biozentrum, University of Basel, Klingelbergstrasse 70, CH 4056, Basel, Switzerland
    Biochim Biophys Acta 1647:206-13. 2003
    ..We have recently solved the crystal structure of a truncated form of this enzyme, which enables us to correlate some of these mutations with the structure...
  13. ncbi request reprint Cystathionine beta-synthase: structure, function, regulation, and location of homocystinuria-causing mutations
    Edith Wilson Miles
    NIDDK, National Institutes of Health, Bethesda, Maryland 20892 0830, USA
    J Biol Chem 279:29871-4. 2004
  14. ncbi request reprint The molecular basis of cystathionine beta-synthase deficiency in Australian patients: genotype-phenotype correlations and response to treatment
    Mette Gaustadnes
    Cardiovascular Genetics Laboratory, Prince of Wales Hospital, Randwick, New South Wales, Australia
    Hum Mutat 20:117-26. 2002
    ..The G307S mutation always resulted in a severe non-responsive phenotype, whereas I278T resulted in a milder B6 responsive phenotype. From our results, we were also able to establish three other mild mutations: P49L, R369C, and V371M...
  15. ncbi request reprint Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli
    Hua Jiang
    Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado 80045, USA
    J Biol Chem 280:27719-27. 2005
    ..The strong favorable effect of the co-expressed chaperone proteins on PCC folding, assembly, and activity suggest that propionic acidemia may be amenable to chaperone therapy...
  16. ncbi request reprint High prevalence of CBS p.T191M mutation in homocystinuric patients from Colombia
    Marta Bermudez
    Instituto de Genetica Humana Pontificia Universidad Javeriana, Bogota, Colombia
    Hum Mutat 27:296. 2006
    ..T191M mutation, a new mutation, p.A288T, was identified in a single individual. In this paper we present the first characterization, at a molecular level, of patients with homocystinuria from Colombia...
  17. pmc Diversity of cystathionine beta-synthase haplotypes bearing the most common homocystinuria mutation c.833T>C: a possible role for gene conversion
    Petr Vyletal
    Center for Applied Genomics, Institute of Inherited Metabolic Disorders, Charles University 1st Faculty of Medicine, Prague, Czech Republic
    Hum Mutat 28:255-64. 2007
    ..833C; -] chromosomes may have originated by recurrent gene conversion employing the common nonpathogenic c.[833C; 844_845ins68] chromosomes as templates...
  18. ncbi request reprint The heme of cystathionine beta-synthase likely undergoes a thermally induced redox-mediated ligand switch
    Samuel Pazicni
    Department of Chemistry, University of Wisconsin Madison, Madison, Wisconsin 53706, USA
    Biochemistry 44:16785-95. 2005
    ....
  19. ncbi request reprint Expression study of mutant cystathionine beta-synthase found in Japanese patients with homocystinuria
    Fumio Katsushima
    Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan
    Mol Genet Metab 87:323-8. 2006
    ..Patient-derived lymphocytes, however, showed no detectable CBS subunits. As previously hypothesized, the increased aggregation of mutant CBS subunits might be a common pathogenic mechanism in CBS deficiency...
  20. ncbi request reprint Inhibitory effect of copper on cystathionine beta-synthase activity: protective effect of an analog of the human albumin N-terminus
    David Bar-Or
    Department of Trauma Research and Trauma Services, Swedish Medical Center, 501 E Hampden Avenue, Englewood, CO 80113, USA
    Protein Pept Lett 12:271-3. 2005
    ..The use of copper chelators could significantly reduce in vivo homocysteine levels...
  21. ncbi request reprint Plasma albumin cysteinylation is regulated by cystathionine beta-synthase
    David Bar-Or
    Trauma Research Department, Swedish Medical Center, Englewood, CO, USA
    Biochem Biophys Res Commun 325:1449-53. 2004
    ..CysAlb may serve as a biomarker for the severity of gut ischemia, and high Hcy may explain endothelial damage associated with this model. Additionally, active CBS is essential for the formation of CysAlb...
  22. ncbi request reprint The molecular basis of cystathionine beta-synthase (CBS) deficiency in UK and US patients with homocystinuria
    Stuart J Moat
    Cardiovascular Sciences Research Group, University of Wales College of Medicine, Heath Park, Cardiff, UK
    Hum Mutat 23:206. 2004
    ..The spectrum of mutations observed in the patients from the UK and US is closer to that which is observed in Northern Europe and bears less resemblance to that observed in Ireland...
  23. ncbi request reprint Hormonal regulation of cystathionine beta-synthase expression in liver
    Shobhitha Ratnam
    Department of Biochemistry, Memorial University of Newfoundland, St John s, Newfoundland A1B 3X9, Canada
    J Biol Chem 277:42912-8. 2002
    ..These results show that insulin has a direct role in regulating homocysteine metabolism. Altered insulin levels in diseases such as diabetes may influence homocysteine metabolism by regulating the hepatic transsulfuration pathway...
  24. ncbi request reprint The redox behavior of the heme in cystathionine beta-synthase is sensitive to pH
    Samuel Pazicni
    Department of Chemistry, University of Wisconsin, Madison, Wisconsin 53706, USA
    Biochemistry 43:14684-95. 2004
    ..Given that the redox behavior of the CBS heme appears to be controlled by pH, interplay of pH and oxidation state effects must occur if CBS activity is redox regulated...

Research Grants2

  1. Homocysteine & Heme: A Role in Thrombosis?
    JAN KRAUS; Fiscal Year: 2009
    ..This project looks at the errors in the CBS protein, and tries to learn from those errors how the protein normally works. ..