Human CBS Matched Antibody Pair set for ELISA use
Capture Antibody: Rabbit purified polyclonal anti-CBS
Detection Antibody: Mouse monoclonal anti-CBS, IgG1 Kappa
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Antigen Detail | |
|---|---|
| Target | CBS |
| Description | Cystathionine-β-synthase, also known as CBS, is an enzyme (EC 4.2.1.22) that in humans is encoded by the CBS gene. It catalyzes the first step of the transsulfuration pathway, from homocysteine to cystathionine. CBS is a multidomain enzyme composed of an N-terminal enzymatic domain and two CBS domains. The CBS gene is the most common locus for mutations associated with homocystinuria. |
| Species Reactivity | Human |
Capture Antibody | |
|---|---|
| Capture Clonality | Polyclonal |
| Host Species | Rabbit |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4, containing 0.1% Proclin 300 |
| Application | ELISA |
Detection Antibody | |
|---|---|
| Det-Clonality | Monoclonal |
| Host Species | Mouse |
| Conjugation | Unconjugated |
| Clone | RD10451 |
| Isotype | IgG1, kappa |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4 |
General Product Property | |
|---|---|
| Product Overview | Human CBS Matched Antibody Pair set for ELISA use Capture Antibody: Rabbit purified polyclonal anti-CBS Detection Antibody: Mouse monoclonal anti-CBS, IgG1 Kappa |
| Specificity | Validated for detecting natural and recombinant human CBS |
| Research Area | Folate Metabolism |
| Application | ELISA |
| Application Notes | Optimal dilutions should be determined experimentally by the user. |
| Form | Lyophilized |
| Storage | Avoid repeated freeze/thaw cycles. Store at 2-8°C for short term. Aliquot and store at -20°C for long term storage. |
| Size | 1 Set |
| Sample Type | Serum; Supernatant; Urine; Cell lysate; Plasma; Tissue Homogenates |
| Usage Statement | Available for Research Use Only. Not for use in therapeutic or diagnostic applications. |
Target Overview | |
|---|---|
| Gene Symbol | CBS |
| Full Name | Cystathionine Beta-Synthase |
| Background | The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016] |
| Alternative Names | Serine Sulfhydrase; Beta-Thionase |
| Gene ID | 875 |
| Uniprot ID | P35520 |
| HGNC | 1550 |
| Ensembl | ENSG00000160200 |
| OMIM | 613381 |
| Involvement in Disease | Diseases associated with CBS include Homocystinuria Due To Cystathionine Beta-Synthase Deficiency and Homocystinuria. |
| Function | Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (PubMed:23981774, PubMed:20506325, PubMed:23974653). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity). |
| Related Pathways | Its related pathways are Folate Metabolism and One carbon pool by folate. |
For Research Use Only.