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Gene Review

CYS4  -  cystathionine beta-synthase CYS4

Saccharomyces cerevisiae S288c

Synonyms: Beta-thionase, Cystathionine beta-synthase, G6667, NHS5, STR4, ...
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Disease relevance of CYS4

  • Mutations in cystathionine beta-synthase (CBS) are known to cause homocystinuria, a recessive disorder characterized by excessive levels of total homocysteine (tHcy) in plasma [1].
  • We report methods that yielded high expression of the yeast gene in Escherichia coli and of purified yeast cystathionine beta-synthase [2].

Psychiatry related information on CYS4

  • Moreover, in the case of the other strain, higher levels of enzymes required for sulfur metabolism (Cys4p, Hom6p, and Met22p) are observed, which could be related to the production of particular organoleptic compounds or to detoxification processes [3].

High impact information on CYS4

  • This can result from heterozygosity at the CBS locus or polymorphic variation in other enzymes involved in homocysteine re-methylation [1].
  • This C-terminal domain of CBS acts to inhibit enzymatic activity and is in turn regulated by S-adenosylmethionine (AdoMet), a positive effector of CBS [1].
  • We report here that a mutation which deletes the carboxy-terminal 145 amino acids of CBS can functionally suppress the phenotype of several CBS mutant alleles found in homocystinurics when expressed in yeast [1].
  • We have isolated the samB gene (suppressor of anucleate metulae) of Aspergillus nidulans which encodes a 66 kDa protein carrying an atypical Cys4 and an additional Cys2/His/Cys Zn finger motif at the carboxy-terminus [4].
  • By screening based on these phenotypes and the inability of vma mutants to accumulate the lysosomotropic dye quinacrine in their vacuoles, five new vma complementation groups (vma41 to vma45) were identified [5].

Chemical compound and disease context of CYS4


Biological context of CYS4


Anatomical context of CYS4

  • In addition, we have used the yeast CBS assay to identify eight mutant CBS alleles in cell lines from patients with CBS deficiency [10].
  • The data indicate that the acidification defect of cys4 mutants arises from inactivation of the vacuolar ATPase in the less reducing cytosol resulting from loss of Cys4p activity and provide the first evidence for the modulation of V-ATPase activity by the redox state of the environment in vivo [7].

Associations of CYS4 with chemical compounds


Other interactions of CYS4


Analytical, diagnostic and therapeutic context of CYS4


  1. Correction of disease-causing CBS mutations in yeast. Shan, X., Kruger, W.D. Nat. Genet. (1998) [Pubmed]
  2. Yeast cystathionine beta-synthase is a pyridoxal phosphate enzyme but, unlike the human enzyme, is not a heme protein. Jhee, K.H., McPhie, P., Miles, E.W. J. Biol. Chem. (2000) [Pubmed]
  3. Transcriptomic and proteomic approach for understanding the molecular basis of adaptation of Saccharomyces cerevisiae to wine fermentation. Zuzuarregui, A., Monteoliva, L., Gil, C., del Olmo, M. Appl. Environ. Microbiol. (2006) [Pubmed]
  4. Integrity of a Zn finger-like domain in SamB is crucial for morphogenesis in ascomycetous fungi. Krüger, M., Fischer, R. EMBO J. (1998) [Pubmed]
  5. Mutations in the yeast KEX2 gene cause a Vma(-)-like phenotype: a possible role for the Kex2 endoprotease in vacuolar acidification. Oluwatosin, Y.E., Kane, P.M. Mol. Cell. Biol. (1998) [Pubmed]
  6. The role of cystathionine beta-synthase in homocysteine metabolism. Jhee, K.H., Kruger, W.D. Antioxid. Redox Signal. (2005) [Pubmed]
  7. Mutations in the CYS4 gene provide evidence for regulation of the yeast vacuolar H+-ATPase by oxidation and reduction in vivo. Oluwatosin, Y.E., Kane, P.M. J. Biol. Chem. (1997) [Pubmed]
  8. Purification and properties of Saccharomyces cerevisiae cystathionine beta-synthase. Ono, B., Kijima, K., Inoue, T., Miyoshi, S., Matsuda, A., Shinoda, S. Yeast (1994) [Pubmed]
  9. Cloning and mapping of the CYS4 gene of Saccharomyces cerevisiae. Ono, B., Heike, C., Yano, Y., Inoue, T., Naito, K., Nakagami, S., Yamane, A. Curr. Genet. (1992) [Pubmed]
  10. A yeast assay for functional detection of mutations in the human cystathionine beta-synthase gene. Kruger, W.D., Cox, D.R. Hum. Mol. Genet. (1995) [Pubmed]
  11. Homocysteine accumulation causes a defect in purine biosynthesis: further characterization of Schizosaccharomyces pombe methionine auxotrophs. Fujita, Y., Ukena, E., Iefuji, H., Giga-Hama, Y., Takegawa, K. Microbiology (Reading, Engl.) (2006) [Pubmed]
  12. Regulation of cystathionine gamma-lyase in Saccharomyces cerevisiae. Ono, B., Naito, K., Shirahige, Y., Yamamoto, M. Yeast (1991) [Pubmed]
  13. Sulphur amino acid synthesis in Schizosaccharomyces pombe represents a specific variant of sulphur metabolism in fungi. Brzywczy, J., Sieńko, M., Kucharska, A., Paszewski, A. Yeast (2002) [Pubmed]
  14. Saccharomyces cerevisiae cultured under aerobic and anaerobic conditions: air-level oxygen stress and protection against stress. Ohmori, S., Nawata, Y., Kiyono, K., Murata, H., Tsuboi, S., Ikeda, M., Akagi, R., Morohashi, K.I., Ono, B. Biochim. Biophys. Acta (1999) [Pubmed]
  15. Cysteine biosynthesis in Saccharomyces cerevisiae: a new outlook on pathway and regulation. Ono, B.I., Hazu, T., Yoshida, S., Kawato, T., Shinoda, S., Brzvwczy, J., Paszewski, A. Yeast (1999) [Pubmed]
  16. Molecular characterization of a cystathionine beta-synthase gene, CBS1, in Magnaporthe grisea. Lo, S.C., Hamer, L., Hamer, J.E. Eukaryotic Cell (2002) [Pubmed]
  17. The reaction of yeast cystathionine beta-synthase is rate-limited by the conversion of aminoacrylate to cystathionine. Jhee, K.H., Niks, D., McPhie, P., Dunn, M.F., Miles, E.W. Biochemistry (2001) [Pubmed]
  18. Cystathionine beta-synthase is coordinately regulated with proliferation through a redox-sensitive mechanism in cultured human cells and Saccharomyces cerevisiae. Maclean, K.N., Janosík, M., Kraus, E., Kozich, V., Allen, R.H., Raab, B.K., Kraus, J.P. J. Cell. Physiol. (2002) [Pubmed]
  19. Trypsin cleavage of human cystathionine beta-synthase into an evolutionarily conserved active core: structural and functional consequences. Kery, V., Poneleit, L., Kraus, J.P. Arch. Biochem. Biophys. (1998) [Pubmed]
  20. Sensitive determination of cystathionine and assays for cystathionine beta- and gamma-lyase, as well as cystathionine beta-synthase, using high-performance liquid chromatography. Ohmori, S., Nakata, K., Nishihara, K., Yamamoto, S., Kawase, M., Tsuboi, S. J. Chromatogr. (1992) [Pubmed]
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