GCH1

Size

£460.00

SKU:: 316965
Purity:: Purified by immunoaffinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide of human GCH1
Format:: Supplied as a liquid in PBS, pH 7.4, 0.05% sodium azide, 40% glycerol.
Extra Details:: 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. Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:1000-1:5000 Western Blot: 1:500-1:2000 Immunohistochemistry: 1:5-1:20 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.