CTH (1p31.1) Kystatiinigammalyaasi

CTH cystathionine gamma-lyase [ Homo sapiens (human) ]

https://www.ncbi.nlm.nih.gov/gene/1491

Summary. 

This gene encodes a cytoplasmic enzyme in the trans-sulfuration pathway (TS)  that converts cystathione (cystat)  derived from methionine (Met) into cysteine (Cys). 

Glutathione (GSH) synthesis in the liver is dependent upon the availability of cysteine. 

Mutations in this gene cause cystathioninuria. Alternative splicing of this gene results in three transcript variants encoding different isoforms. [provided by RefSeq, Jun 2010]

Expression Biased expression in liver (RPKM 17.3), adrenal (RPKM 4.0) and 12 other tissues See more Orthologs mouse all

Preferred Names

cystathionine gamma-lyase

Names

cystathionase (cystathionine gamma-lyase)

cysteine desulfhydrase

cysteine-protein sulfhydrase

gamma-cystathionase

homoserine deaminase

homoserine dehydratase

Related

ENSP00000413407.2, ENST00000411986.6

Conserved Domains (2) summary

PRK09028
Location:19 → 347

PRK09028; cystathionine beta-lyase; Provisional

pfam01053
Location:19 → 347

Cys/Met metabolizm PLP-dependent (B6 vitamin)  enzyme

This family includes enzymes involved in cysteine and methionine metabolizm. The following are members: Cystathionine gamma-lyase, Cystathionine gamma-synthase, Cystathionine beta-lyase, Methionine gamma-lyase, OAH/OAS sulfhydrylase, O-succinylhomoserine sulfhydrylase All of these members participate is slightly different reactions. All these enzymes use PLP (pyridoxal-5'-phosphate) as a cofactor.

Related articles in PubMed

1. Total sulfane sulfur bioavailability reflects ethnic and gender disparities in cardiovascular disease. Rajpal S, et al. Redox Biol, 2018 May. PMID 29413960, Free PMC Article
2. Age-Dependent Allergic Asthma Development and Cystathionine Gamma-Lyase Deficiency. Wang P, et al. Antioxid Redox Signal, 2017 Nov 1. PMID 28253731 Abstract AIMS:
   The pathogenic mechanisms for the higher prevalence of allergic asthma in children than in adults have not been settled. The aim of the present study is to examine whether the age-dependent development of allergic asthma is caused by age-dependent expression of cystathionine gamma-lyase (CSE), a key enzyme that catalyzes the production of hydrogen sulfide (H₂S). RESULTS:
   Allergic asthma was induced with ovalbumin in wild-type (WT) and CSE knock-out (KO) mice at young and old ages. CSE expression and H₂S production were lower in immune cells of young WT mice than in those of old WT mice. Coincidentally, more severe asthmatic symptoms with a greater type-2 immunoreaction were found in young WT mice than old WT mice. H₂S supplementation reversed the asthmatic symptoms. Lower expression levels of CSE proteins were also found in human umbilical cord blood mononuclear cells in comparison with that of peripheral blood mononuclear cells from adult people. The age-dependent asthma propensity vanished in CSE-KO mice, but these mice developed more severe asthma than WT mice. More splenocytes were differentiated to type-2 cytokine-generating cells in young WT mice and in CSE-KO mice at all ages. This differentiation was inhibited by H₂S donors. GATA3 translocation to the nucleus and type-2 immunoreaction of splenocytes were inhibited after GATA3 was S-sulfhydrated by H₂S.
   ( Kommenttitti: GATAD ovat sinkkisormiproteiineja, joilla on C-x-C-x-C-x-C  sinkkisormirakenne ja ne ovat transkriptiotekijöiotä) .
   Innovation and Conclusion: For the first time, this study demonstrated that lower abundance of CSE expression and H₂S production enhances type-2 immunoreaction and renders a higher incidence of allergic asthma at a young age. As such, H₂S level may be a biomarker for asthma development and a H₂S-based strategy can be perceived for asthma prevention and treatment. Antioxid. Redox Signal. 27, 931-944.
3. Dichotomous effects of isomeric secondary amines containing an aromatic nitrile and nitro group on human aortic smooth muscle cells via inhibition of cystathionine-γ-lyase. Ji Y, et al. Biochimie, 2017 Feb. PMID 28034716, Free PMC Article
4. Cystathionine γ-lyase is expressed in human atherosclerotic plaque microvessels and is involved in micro-angiogenesis. van den Born JC, et al. Sci Rep, 2016 Oct 6. PMID 27708362, Free PMC Article
5. Radiation Exposure Promotes Hepatocarcinoma Cell Invasion through Epithelial Mesenchymal Transition Mediated by H2S/CSE Pathway. Pan Y, et al. Radiat Res, 2016 Jan. PMID 26727544
   Radiat Res. 2016 Jan;185(1):96-105. doi: 10.1667/RR14177.1. Epub 2016 Jan 4.
   Radiation Exposure Promotes Hepatocarcinoma Cell Invasion through Epithelial Mesenchymal Transition Mediated by H2S/CSE Pathway.
   
   Pan Y¹, Zhou C¹, Yuan D¹, Zhang J¹, Shao C¹.Abstract
   There is growing evidence to suggest that radiotherapy can paradoxically promote tumor invasion and metastatic processes, however, the underlying molecular mechanisms remain obscure. In this study, we found that exposure to X rays promoted cell invasion by triggering the epithelial mesenchymal transition (EMT) in two hepatocellular carcinoma (HCC) cell lines, HepG2 and PLC/PRF/5. This was made evident by a reduced expression of E-cadherin and enhanced expressions of N-cadherin, Vimentin and Snail.
   Moreover, exposure to radiation stimulated the signaling of hydrogen sulfide (H2S), a newly found gas transmitter, by upregulating the expressions of H2S-producing proteins of cysthionine-γ-lyase (CSE), cystathionine-β-synthase (CBS). Inhibition of CSE by siRNA or inhibitor not only increased the radiosensitivity but also strongly suppressed radiation-enhanced invasive properties of HCC cells. Interestingly, we found that H2S/CSE inhibition attenuated radiation-enhanced EMT, and the above effect was an end result of blockage of the radiation-activated pathway of p38 mitogen-activated protein kinase (p38MAPK). Collectively, our findings indicate that radiation could promote HCC cell invasion through EMT mediated by endogenous H2S/CSE signaling via the p38MAPK pathway.

See all (87) citations in PubMed