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FLASH GENE
Symbol CTH contributors: mct - updated : 03-07-2018
HGNC name cystathionase (cystathionine gamma-lyase)
HGNC id 2501
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
mono polymer homomer , tetramer
HOMOLOGY
Homologene
FAMILY trans-sulfuration enzymes family
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,cytosolic
intracellular,nucleus
basic FUNCTION
  • involved in the final step in the trans-sulfuration pathway synthesizing L-cysteine from L-methionine
  • key enzyme in the trans-sulfuration pathway, which uses L-cysteine to produce hydrogen sulfide (H2S)
  • critical for cysteine biosynthesis through the transsulfuration pathway and the combination of CTH deficiency and lack of dietary cysteine supply would threaten life sustainability
  • one of the major enzymes for the production of hydrogen sulphide (H(2)S), a multifunctional gasotransmitter in the pancreatic beta-cell
  • although CTH is responsible for the H2S production in the heart, CTH proteins are undetectable in cardiomyocytes or heart tissues
  • protects likely against renal ischemia/reperfusion injury, likely by modulating oxidative stress through the production of H2S
  • is a key enzyme in the trans-sulfuration pathway responsible for the production of endogenous H2S
  • CBS and CTH are critical for maintenance of mitochondrial function and glucocorticoid production in adrenal cortex
  • is one major H2S-producing enzyme with L-cysteine as the main substrate in mammalian cells
  • the l-cysteine/CTH/H2 S pathway is involved in melanoma progression.
  • key role for the H2S-generating enzymes CBS and CTH in pulmonary vascular development and homeostasis and in lung alveolarization
  • is expressed in human atherosclerotic plaque microvessels and is involved in micro-angiogenesis
  • last key enzyme of the transsulfuration pathway, involved in the production of hydrogen sulfide (H2S) and glutathione (GSH), which regulate redox balance and act as important antioxidant molecules
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding, cofactor,
    pyridoxal-5'-phosphate
    protein
  • SP1 is a critical regulator of the CTH expression during smooth muscle cells differentiation
  • Ca(2+)-dependent CTH expression may be mediated via protein phosphorylation of SP1 and ELK1 in pancreatic beta-cells
  • NOX4 is a positive transcriptional regulator of CTH in endothelial cells and propose that it may in turn contribute to the regulation of vascular tone via the modulation of H2S production
  • cell & other
    REGULATION
    activated by physiologically activated by calcium-calmodulin, which is a mechanism for H2S formation in response to vascular activation
    Other in prostatic tissue, its expression were modulated by dihydrotestosterone
    can respond to hypoxia through transcriptional and post-transcriptional regulation, and CTH expression can be up-regulated by hypoxia to a certain extent
    ASSOCIATED DISORDERS
    corresponding disease(s) CTHD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    stimulates smooth muscle cells apoptosis due to an increased endogenous production of H2S
    constitutional   deletion    
    CTH gene deletion exacerbated mitochondrial fragmentation and apoptosis of renal tubule cells after unilateral ureteral obstruction (UUO)
    Susceptibility to atherosclerosis
    Variant & Polymorphism SNP 1364T/T associated with higher homocysteine concentration and risk of atherosclerosis
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS