127227-BIOTIN
GCH1 (GTP Cyclohydrolase 1, GTP Cyclohydrolase I, GTP-CH-I, DYT5, GCH) (Biotin)
Size
£914.00
- SKU:
- 127227-BIOTIN
- Purity:
- Purified
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Partial recombinant protein corresponding to aa84-172 from human GCH1 (NP_000152) with GST tag. MW of the GST tag alone is 26kD.
- Clone:
- 4A12
- Format:
- Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
- 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, Immunoprecipitation and Immunohistochemistry. Other applications not tested. Recommended Dilutions: Immunohistochemistry (FFPE): 3ug/ml Optimal dilutions to be determined by the researcher. Amino Acid Sequence: ENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIV Storage and Stability: Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Note: Applications are based on unconjugated antibody.
- Shipping Conditions:
- Blue Ice