Human GCH1 Matched Antibody Pair set for ELISA use
Capture Antibody: Mouse monoclonal anti-GCH1
Detection Antibody: Rabbit purified polyclonal anti-GCH1
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Antigen Detail | |
|---|---|
| Target | GCH1 |
| Description | GTP cyclohydrolase I (GTPCH) (EC 3.5.4.16) is a member of the GTP cyclohydrolase family of enzymes. GTPCH is part of the folate and biopterin biosynthesis pathways. It is responsible for the hydrolysis of guanosine triphosphate (GTP) to form 7,8-dihydroneopterin triphosphate (7,8-DHNP-3'-TP, 7,8-NH2-3'-TP). Mutations in this gene are associated with malignant phenylketonuria (PKU) and hyperphenylalaninemia (HPA), as well as GTP cyclohydrolase I deficiency. |
| Species Reactivity | Human |
Capture Antibody | |
|---|---|
| Capture Clonality | Monoclonal |
| Host Species | Mouse |
| Clone | IV01897 |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4, containing 0.1% Proclin 300 |
| Application | ELISA |
Detection Antibody | |
|---|---|
| Det-Clonality | Polyclonal |
| Host Species | Rabbit |
| Conjugation | Unconjugated |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4 |
General Product Property | |
|---|---|
| Product Overview | Human GCH1 Matched Antibody Pair set for ELISA use Capture Antibody: Mouse monoclonal anti-GCH1 Detection Antibody: Rabbit purified polyclonal anti-GCH1 |
| Specificity | Validated for detecting natural and recombinant human GCH1 |
| Research Area | Neurotransmitter |
| 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 | GCH1 |
| Full Name | GTP cyclohydrolase I |
| Background | This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme. [provided by RefSeq, Jul 2008] |
| Alternative Names | GTP-CH-I |
| Gene ID | 2643 |
| Uniprot ID | P30793 |
| GeneCards | GCH1 |
| HGNC | 4193 |
| Ensembl | ENSG00000131979 |
| OMIM | 600225 |
| Involvement in Disease | Diseases associated with GCH1 include Dystonia, Dopa-Responsive and Hyperphenylalaninemia, Bh4-Deficient, B. |
| Function | Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown. |
| Related Pathways | Its related pathways are eNOS activation and regulation and Biosynthesis of cofactors. |
For Research Use Only.