Human Cystathionine gamma-Lyase (CTH) ELISA Kit | CTH elisa kit
Human Cystathionine gamma-Lyase (CTH) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Background: CSE is an enzyme which breaks down cystathionine into cysteine and alpha-ketobutyrate. Pyridoxal phosphate is a prosthetic group of this enzyme.The bioavailability of cysteine is rate-limiting for the hepatic synthesis of glutathione. Cysteine is provided through either the diet or the transsulfuration pathway, in which cystathionine derived from methionine is transformed into cysteine by cystathionine gamma-lyase.Mutant mice lacking CTH displayed pronounced hypertension and diminished endothelium-dependent vasorelaxation. Cystathionine was physiologically activated by calcium and calmodulin, which is a mechanism for H2S formation in response to vascular activation. Yang et al. (2008) concluded that their findings provided direct evidence that H2S is a physiologic vasodilator and regulator of blood pressure.
NCBI and Uniprot Product Information
NCBI Description
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 Description
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; Energy Metabolism - nitrogen; Amino Acid Metabolism - glycine, serine and threonine; Other Amino Acids Metabolism - selenoamino acid; Cell cycle regulation; EC 4.4.1.1; Lyase
Chromosomal Location of Human Ortholog: 1p31.1
Cellular Component: nucleoplasm; cytoplasm; nucleus; cytosol
Molecular Function: calmodulin binding; protein binding; cystathionine gamma-lyase activity; carbon-sulfur lyase activity; homocysteine desulfhydrase 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; transsulfuration; sulfur amino acid catabolic process; cysteine biosynthetic process; activation of NF-kappaB transcription factor; protein homotetramerization
Disease: Cystathioninuria
Research Articles on CTH
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Product Notes
The Human CTH cth (Catalog #AAA9715931) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9715931 ELISA Kit recognizes Human CTH. It is sometimes possible for the material contained within the vial of "Cystathionine gamma-Lyase (CTH), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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