GCH1 / Unconjugated /
GCH1 / Unconjugated /
Product Details
Supplier | LifeSpan | |
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Catalog #: | LS-C135566 (View supplier product page) | |
Size | 100 μl | |
Price | $457.00 | |
Antigen | GCH1 | |
Clone | ||
Host | Rabbit | |
Isotype | IgG | |
Conjugate | Unconjugated | |
Target Species | Bat, Bovine, Canine, Chicken, Equine, Guinea Pig, Hamster, Human, Monkey, Mouse, Porcine, Rabbit, Rat, Xenopus | |
Applications | WB | |
Description | GTPCH1 / GCH1 Antibody (C-Terminus) |
About GCH1 and Purified
GCH1 | 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] |
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