Price range: $268.00 through $328.00
Background:
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],
| Size | 50μL/100μL |
| Target | Cystathionase |
| Reactivity | Human, Mouse |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 44kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000; ELISA 1:5000-20000 |
| Formulation | Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from part region of human protein |
| Specificity | Cystathionase Polyclonal Antibody detects endogenous levels of protein. |
| Gene Name | CTH |
| Protein Name | Cystathionine gamma-lyase (Cysteine-protein sulfhydrase) (Gamma-cystathionase) |
| Other Name | |
| Species | Human |
| Gene ID | 1491 |
| Uniprot | P32929, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q8VCN5, |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | P18757 |
| Cellular Localization | Cytoplasm. |
| Tissue Expression | Highly expressed in liver (PubMed:10727430, PubMed:20305127). Also in muscle and lower expression in most tissues except heart, pituitary gland, spleen, thymus, and vascular tissue, where it is hardly detected (PubMed:20305127). |
| Signaling_pathway | |
| Research Areas | >>Glycine, serine and threonine metabolism; / >>Cysteine and methionine metabolism; / >>Selenocompound metabolism; / >>Metabolic pathways; / >>Biosynthesis of amino acids |
| Function | Catalytic activity:L-cystathionine + H(2)O = L-cysteine + NH(3) + 2-oxobutanoate.,cofactor:Pyridoxal phosphate.,Disease:Defects in CTH are the cause of cystathioninuria [MIM:219500]. CTH is an autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion.,pathway:Amino-acid biosynthesis; L-cysteine biosynthesis; L-cysteine from L-homocysteine and L-serine: step 2/2.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the trans-sulfuration enzymes family.,subunit:Homotetramer., |
