catalog number :
MBS7047965
products full name :
CTH Antibody
products short name :
[CTH]
products name syn :
[Cystathionine gamma-lyase; Cysteine-protein sulfhydrase; Gamma-cystathionase; CTH]
other names :
[cystathionine gamma-lyase isoform 3; Cystathionine gamma-lyase; cystathionine gamma-lyase; cystathionine gamma-lyase; Cysteine-protein sulfhydrase; Gamma-cystathionase]
products gene name :
[CTH]
other gene names :
[CTH; CTH]
uniprot entry name :
CGL_HUMAN
reactivity :
Human, Mouse, Rat
purity :
>95%, Protein G purified
storage stability :
Upon receipt, store at -20 degree C or -80 degree C. Avoid repeated freeze.
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
app notes :
Recommended Dilution: . WB: 1: 500-1: 5000. IHC: 1: 20-1: 200. IF: 1: 50-1: 200
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: Recombinant Human Cystathionine gamma-lyase protein (1-70AA)
other info2 :
Preservative: 0.03% Proclin 300. Constituents: 50% Glycerol, 0.01M PBS, pH 7.4. Conjugation: Non-conjugated. Species: Human. Research Area: Signal Transduction. Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-101924 / sc-131905
products description :
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.
ncbi acc num :
NP_001177392.1
ncbi gb acc num :
NM_001190463.1
ncbi pathways :
Amino Acid Metabolism Pathway (1471249); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Cysteine And Methionine Metabolism Pathway (104488); Cysteine And Methionine Metabolism Pathway (103421); Cysteine Biosynthesis, Homocysteine + Serine = Cysteine Pathway (413414); Cysteine Biosynthesis, Homocysteine + Serine = Cysteine Pathway (468303); Cysteine Formation From Homocysteine Pathway (1270182); Degradation Of Cysteine And Homocysteine Pathway (1270183); Folate Metabolism Pathway (198833)
ncbi summary :
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 summary :
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 - cysteine and methionine; Amino Acid Metabolism - glycine, serine and threonine; Cell cycle regulation; EC 4.4.1.1; Energy Metabolism - nitrogen; Lyase; Other Amino Acids Metabolism - selenoamino acid. Chromosomal Location of Human Ortholog: 1p31.1. Cellular Component: cytoplasm; cytosol; nucleus. Molecular Function: carbon-sulfur lyase activity; cystathionine gamma-lyase activity; cystathionine gamma-synthase activity; homocysteine desulfhydrase activity; identical protein binding; protein binding; pyridoxal phosphate binding. Biological Process: cysteine biosynthetic process; cysteine biosynthetic process via cystathionine; cysteine metabolic process; protein homotetramerization; protein-pyridoxal-5-phosphate linkage via peptidyl-N6-pyridoxal phosphate-L-lysine; sulfur amino acid catabolic process; transsulfuration; unfolded protein response. Disease: Cystathioninuria