Host: Rabbit

Target Protein: GCH1

IR: Immunogen Range:101-200/250

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 2643

Swiss Prot: P30793

Source: KLH conjugated synthetic peptide derived from human GCH1:101-200/250 

Purification: affinity purified by Protein A

Storage: 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.  Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

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. [provided by RefSeq, Jul 2008]