| CatalogCode: | H00002643-M01 |
| ProductName: | GCH1 Antibody |
| Product Description: | Mouse Monoclonal anti-GCH1 (4A12) |
| Clone: | 4A12 |
| Clonality: | Monoclonal |
| Immunogen: | GCH1 (NP_000152, 84 a.a. ~ 173 a.a) partial recombinant protein with GST tag. |
| Epitope: | ENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIV* ... |
| Specificity: | GCH1 - GTP cyclohydrolase 1 (dopa-responsive dystonia) |
| CrossReactivity: | Human. Other species not tested. |
| Packaging: | 0.1 mg IgG purified Mouse ascites. |
| Uses: | Antibody Reactive against Recombinant Protein with GST tag on ELISA and Western Blot. GST tag alone is used as a negative control. The antibody is also shown to be specific in Western blot against cell lysates. It has also been used in immunohistochemistry on paraffin-embedded sections. |
| 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. |
| ProductRef: | Regulation of Tetrahydrobiopterin Biosynthesis by Shear Stress.Widder JD, Harrison DG. et al. Circ Res. 2007 Aug 17; [Epub ahead of print] Abnova product used: |
| Storage: | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| Purity: | IgG purified |
| Isotype: | IgG2a Kappa |
| Host_Name: | Mouse |
| ListPrice: | 295 |
| AppSummary: | ELISA, WB, IHC-P |
| SpeciesSummary: | Hu |
| ALTnames: | anti-DYT5 antibody, anti-GCH antibody, anti-GTP-CH-1 antibody, anti-GTPCH1 antibody |
| ProteinTarget: | GCH1 - GTP cyclohydrolase 1 (dopa-responsive dystonia) |
| PackageSize: | 0.1 mg |
| NotesMain: | This antibody is available only in North America and is supplied by Abnova If in stock in Taiwan. delivery is 10-14 days. If not in stock. lead times can be extended. We will inform you of any extended delays within 48 hours. The immunizing protein is also available for this product. Unit cost for the protein is $ 250 (2 ug). $ 305 (10 ug). and $ 445 (25 ug). |
| Gene_Id: | 2643 |
| Gene_Symbol: | GCH1 |
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