NP_001177392.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
Predicted Molecular Mass: 28.1kDa Accurate Molecular Mass: 26kDa as determined by SDS-PAGE reducing conditions.
NCBI Official Full Name
cystathionine gamma-lyase isoform 3
NCBI Official Synonym Full Names
cystathionase (cystathionine gamma-lyase)
NCBI Protein Information
cystathionine gamma-lyase; gamma-cystathionase; homoserine deaminase; cysteine desulfhydrase; homoserine dehydratase; cysteine-protein sulfhydrase
UniProt Protein Name
Cystathionine gamma-lyase
UniProt Synonym Protein Names
Cysteine-protein sulfhydrase; Gamma-cystathionase
UniProt Entry Name
CGL_HUMAN
NCBI Summary for CTH
This gene encodes a cytoplasmic enzyme in the trans-sulfuration pathway that converts cystathione derived from methionine into cysteine. Glutathione 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]
UniProt Comments for CTH
CTH: Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function. Defects in CTH are the cause of cystathioninuria (CSTNU). It is an autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion. Belongs to the trans-sulfuration enzymes family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - glycine, serine and threonine; Cell cycle regulation; EC 4.4.1.1; Energy Metabolism - nitrogen; Other Amino Acids Metabolism - selenoamino acid; Amino Acid Metabolism - cysteine and methionine; Lyase
Chromosomal Location of Human Ortholog: 1p31.1
Cellular Component: nucleoplasm; cytoplasm; nucleus; cytosol
Molecular Function: calmodulin binding; protein binding; cystathionine gamma-lyase activity; homocysteine desulfhydrase activity; carbon-sulfur lyase activity; pyridoxal phosphate binding
Biological Process: cysteine metabolic process; positive regulation of I-kappaB kinase/NF-kappaB cascade; unfolded protein response; sulfur amino acid metabolic process; protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine; sulfur amino acid catabolic process; transsulfuration; cysteine biosynthetic process; protein homotetramerization; activation of NF-kappaB transcription factor
Disease: Cystathioninuria
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Products associated with CTH recombinant protein
Pathways associated with CTH recombinant protein
Diseases associated with CTH recombinant protein
Organs/Tissues associated with CTH recombinant protein
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