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Gene Review

CTH  -  cystathionine gamma-lyase

Homo sapiens

Synonyms: Cystathionine gamma-lyase, Cysteine-protein sulfhydrase, Gamma-cystathionase
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Disease relevance of CTH


High impact information on CTH


Chemical compound and disease context of CTH

  • These findings suggest that (1) endogenous H2S is produced by myocardial ischemia in sufficient amounts to limit myocardial injury and (2) the synthesis or formation of H2S by cystathionine-gamma-lyase may contribute to the second window of protection caused by endotoxin [10].

Biological context of CTH


Anatomical context of CTH


Associations of CTH with chemical compounds


Other interactions of CTH


Analytical, diagnostic and therapeutic context of CTH

  • Mutants T6 and T24, obtained by gene replacement, showed an inactive mecB gene by Southern blot analysis and no cystathionine-gamma-lyase activity [25].
  • In another set of experiments, administration of DL-propargylglycine (inhibitor of cystathionine gamma-lyase) to rats resulted in significant reduction in cavernous nerve stimulation-evoked perfusion pressure [26].
  • A mouse monoclonal antibody has been produced which recognizes human liver gamma-cystathionase, Radioiodination of the monoclonal antibody facilitated its use in combination with non-specific precipitating rabbit antisera in classical immunodiffusion assays [27].


  1. Genomic basis of cystathioninuria (MIM 219500) revealed by multiple mutations in cystathionine gamma-lyase (CTH). Wang, J., Hegele, R.A. Hum. Genet. (2003) [Pubmed]
  2. Subpopulations of B cells in germinal centers. III. HJ6, a monoclonal antibody, binds globoside and a subpopulation of germinal center B cells. Madassery, J.V., Gillard, B., Marcus, D.M., Nahm, M.H. J. Immunol. (1991) [Pubmed]
  3. Fabry disease: detection of heterozygotes by examination of glycolipids in urinary sediment. Cable, W.J., McCluer, R.H., Kolodny, E.H., Ullman, M.D. Neurology (1982) [Pubmed]
  4. Unilateral cryptorchidism with compensatory hypertrophy of descended testicle in prepubertal boys. Tatò, L., Corgnati, A., Boner, A., Pinelli, L., Zatti, M., Gaburro, D. Horm. Res. (1978) [Pubmed]
  5. Maternal gamma-cystathionase deficiency: absence of both teratogenic effects and pregnancy complications. Vargas, J.E., Mudd, S.H., Waisbren, S.E., Levy, H.L. Am. J. Obstet. Gynecol. (1999) [Pubmed]
  6. Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Stabler, S.P., Lindenbaum, J., Savage, D.G., Allen, R.H. Blood (1993) [Pubmed]
  7. Imparied glutathione biosynthesis in cultured human ataxia-telangiectasia cells. Meredith, M.J., Dodson, M.L. Cancer Res. (1987) [Pubmed]
  8. Reduced gamma-cystathionase protein content in human malignant leukemia cell lines as measured by immunoassay with monoclonal antibody. Glode, L.M., Epstein, A., Smith, C.G. Cancer Res. (1981) [Pubmed]
  9. Protective effect of hydrogen sulfide on balloon injury-induced neointima hyperplasia in rat carotid arteries. Meng, Q.H., Yang, G., Yang, W., Jiang, B., Wu, L., Wang, R. Am. J. Pathol. (2007) [Pubmed]
  10. The production of hydrogen sulfide limits myocardial ischemia and reperfusion injury and contributes to the cardioprotective effects of preconditioning with endotoxin, but not ischemia in the rat. Sivarajah, A., McDonald, M.C., Thiemermann, C. Shock (2006) [Pubmed]
  11. Kinetics and inhibition of recombinant human cystathionine gamma-lyase. Toward the rational control of transsulfuration. Steegborn, C., Clausen, T., Sondermann, P., Jacob, U., Worbs, M., Marinkovic, S., Huber, R., Wahl, M.C. J. Biol. Chem. (1999) [Pubmed]
  12. Characterization of cystathionine synthase as a selectable, liver-specific trait in rat hepatomas. Goss, S.J. J. Cell. Sci. (1986) [Pubmed]
  13. Identification of the active-site residue of gamma-cystathionase labeled by the suicide inactivator beta, beta, beta-trifluoroalanine. Fearon, C.W., Rodkey, J.A., Abeles, R.H. Biochemistry (1982) [Pubmed]
  14. Human cystathionine gamma-lyase: developmental and in vitro expression of two isoforms. Levonen, A.L., Lapatto, R., Saksela, M., Raivio, K.O. Biochem. J. (2000) [Pubmed]
  15. Glycosphingolipids of purified human lymphocytes. Stein, K.E., Marcus, D.M. Biochemistry (1977) [Pubmed]
  16. Measurement of urinary CDH and CTH by tandem mass spectrometry in patients hemizygous and heterozygous for Fabry disease. Mills, K., Morris, P., Lee, P., Vellodi, A., Waldek, S., Young, E., Winchester, B. J. Inherit. Metab. Dis. (2005) [Pubmed]
  17. New roles for cysteine and transsulfuration enzymes: production of H2S, a neuromodulator and smooth muscle relaxant. Dominy, J.E., Stipanuk, M.H. Nutr. Rev. (2004) [Pubmed]
  18. Hydrogen sulfide and its possible roles in myocardial ischemia in experimental rats. Zhu, Y.Z., Wang, Z.J., Ho, P., Loke, Y.Y., Zhu, Y.C., Huang, S.H., Tan, C.S., Whiteman, M., Lu, J., Moore, P.K. J. Appl. Physiol. (2007) [Pubmed]
  19. Maternal methionine adenosyltransferase I/III deficiency: reproductive outcomes in a woman with four pregnancies. Mudd, S.H., Tangerman, A., Stabler, S.P., Allen, R.H., Wagner, C., Zeisel, S.H., Levy, H.L. J. Inherit. Metab. Dis. (2003) [Pubmed]
  20. Single nucleotide polymorphism in CTH associated with variation in plasma homocysteine concentration. Wang, J., Huff, A.M., Spence, J.D., Hegele, R.A. Clin. Genet. (2004) [Pubmed]
  21. Pro-apoptotic effect of endogenous H2S on human aorta smooth muscle cells. Yang, G., Wu, L., Wang, R. FASEB J. (2006) [Pubmed]
  22. Hydrogen sulfide mediates hypoxia-induced relaxation of trout urinary bladder smooth muscle. Dombkowski, R.A., Doellman, M.M., Head, S.K., Olson, K.R. J. Exp. Biol. (2006) [Pubmed]
  23. Novel mutation in DGUOK in hepatocerebral mitochondrial DNA depletion syndrome associated with cystathioninuria. Tadiboyina, V.T., Rupar, A., Atkison, P., Feigenbaum, A., Kronick, J., Wang, J., Hegele, R.A. Am. J. Med. Genet. A (2005) [Pubmed]
  24. Interaction between hydrogen sulfide/cystathionine gamma-lyase and carbon monoxide/heme oxygenase pathways in aortic smooth muscle cells. Jin, H.F., Du, J.B., Li, X.H., Wang, Y.F., Liang, Y.F., Tang, C.S. Acta Pharmacol. Sin. (2006) [Pubmed]
  25. Targeted inactivation of the mecB gene, encoding cystathionine-gamma-lyase, shows that the reverse transsulfuration pathway is required for high-level cephalosporin biosynthesis in Acremonium chrysogenum C10 but not for methionine induction of the cephalosporin genes. Liu, G., Casqueiro, J., Bañuelos, O., Cardoza, R.E., Gutiérrez, S., Martín, J.F. J. Bacteriol. (2001) [Pubmed]
  26. Possible role for the novel gasotransmitter hydrogen sulphide in erectile dysfunction--a pilot study. Srilatha, B., Adaikan, P.G., Moore, P.K. Eur. J. Pharmacol. (2006) [Pubmed]
  27. Use of monoclonal antibody to increase sensitivity and specificity in quantitative immunodiffusion assays. Glode, L.M., Montgomery, R.R., Smith, C.G., Link, D.B. J. Immunol. Methods (1982) [Pubmed]
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