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/mlOptimal dilutions to be determined by the researcher.
AA Sequence:MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
Storage and Stability:May be stored at 4℃ for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20℃.
Aliquots are stable for at least 12 months.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ug
抗体種類：Pab
宿主：mouse
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2.
Specificity：Recognizes human GCH1.
Isotype：IgG
Calc Applications Abbrev：IF WB
Calc Crossreactivity：Hu
Immunogen：Full length human GCH1, aa1-250 (NP_000152.1).
