G9005-98B
GCH1 (GTP Cyclohydrolase 1, GTP Cyclohydrolase I, GTP-CH-I, DYT5, GCH)
Size
£642.00
- SKU:
- G9005-98B
- Purity:
- Purified by immunoaffinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Goat
- Immunogen:
- Synthetic peptide corresponding to C-GKVHIGYLPNKQ, from human GCH1, at the internal region of the protein (NP_000152.1, NP_001019195.1).
- Format:
- Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.
- 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 and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:16,000 Western Blot: 0.5-1.5ug/ml, observed in human tonsil, lymph node and thymus lysates. on ~37kD bands 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.
- Shipping Conditions:
- Blue Ice