127225
GCH1 (GTP Cyclohydrolase 1, GTP Cyclohydrolase I, GTP-CH-I, DYT5, GCH)
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£685.00
- SKU:
- 127225
- Reinheit:
- Purified by Protein A affinity chromatography.
- Lagerbedingungen:
- -20°C
- Hersteller:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Full length human GCH1, aa1-250 (NP_000152.1).
- Format:
- Supplied as a liquid in PBS, pH 7.2.
- Weitere 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 Immunofluorescence and Western Blot. Other applications not tested. Recommended Dilution: Immunofluorescence: 10ug/ml Optimal dilutions to be determined by the researcher. AA Sequence: MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS 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.
- Versandbedingungen:
- Blue Ice