GTP cyclohydrolase 1 (GCH1) Rabbit Polyclonal Antibody
Other products for "GCH1"
Specifications
Product Data | |
Applications | WB |
Recommended Dilution | WB |
Reactivities | Human |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | The immunogen for anti-GCH1 antibody: synthetic peptide directed towards the C terminal of human GCH1. Synthetic peptide located within the following region: LRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLT |
Formulation | Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers. |
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 |
Note | Immunogen Sequence Homology: Dog: 100%; Pig: 100%; Rat: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Bovine: 100%; Rabbit: 100%; Guinea pig: 100%; Zebrafish: 93%; Yeast: 85% |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Folate biosynthesis, Metabolic pathways |
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complexities in the preparation of your product. International customers may expect an additional 1-2 weeks
in shipping.