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GTP cyclohydrolase 1 (GCH1) Rabbit Polyclonal Antibody
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Specifications
| Product Data | |
| Applications | IHC, WB |
| Recommended Dilution | ELISA: 1:1000-2000, WB: 1:200-1000, IHC: 1:5-20 |
| Reactivities | Human, Mouse, Rat |
| Host | Rabbit |
| Isotype | IgG |
| Clonality | Polyclonal |
| Immunogen | Synthetic peptide corresponding to a region derived from 51-64 amino acids of human GTP cyclohydrolase 1 |
| Formulation | PBS pH7.3, 0.05% NaN3, 50% glycerol |
| Concentration | lot specific |
| Purification | Antigen affinity purification |
| Conjugation | Unconjugated |
| Storage | Store at -20°C as received. |
| Stability | Stable for 12 months from date of receipt. |
| Predicted Protein Size | 28 kDa |
| Gene Name | GTP cyclohydrolase 1 |
| Database Link | |
| 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. |
| Synonyms | DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B |
| Reference Data | |
| Protein Families | Druggable Genome |
| Protein Pathways | Folate biosynthesis, Metabolic pathways |
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