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| Reactivity: | Human |
| Applications: | WB |
| Host Species: | Mouse |
| Isotype: | IgG2a |
| Clonality: | Monoclonal antibody |
| Gene Name: | GTP cyclohydrolase 1 |
| Gene Symbol: | GCH1 |
| Synonyms: | GCH; DYT5; DYT14; DYT5a; GTPCH1; HPABH4B; GTP-CH-1 |
| Gene ID: | 2643 |
| UniProt ID: | P30793 |
| Clone ID: | 24GB13125 |
| Immunogen: | Recombinant protein of human GCH1 |
| Dilution: | WB 1:500-1:2,500 |
| Purification Method: | Affinity purified |
| Concentration: | Lot dependent |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
Background
This gene GCH1 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.
Images
 | Western blotting analysis using anti-GCH1 antibody (Cat#64824). Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with anti-GCH1 antibody (Cat#64824, 1:2,500) and HRP-conjugated goat anti-mouse secondary antibody (Cat#101, 1:20,000) respectively. Image was developed using NaQ™ ECL Substrate Kit (Cat#716). |
 | Western blotting analysis using anti-GCH1 antibody (Cat#64824). GCH1 expression in wild-type (WT) and GCH1 shRNA knockdown (KD) HeLa cells with 30 μg of total cell lysates. Hsp90 α serves as a loading control. The blot was incubated with anti-GCH1 antibody (Cat#64824, 1:2,500) and HRP-conjugated goat anti-mouse secondary antibody (Cat#101, 1:20,000) respectively. Image was developed using FeQ™ ECL Substrate Kit (Cat#226). |
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