246519-APC

GCH1 (GTP Cyclohydrolase 1, DYT14, DYT5, GCH, GTP-CH-1, GTPCH1) (APC)

Size

£914.00

SKU:: 246519-APC
Purity:: Purified
Storage Conditions:: 4°C Do Not Freeze
Supplier:: United States Biological
Host:: Mouse
Immunogen:: GCH1 (AAH25415.1, 1aa-250aa) full-length recombinant protein with GST tag. MW of the GST tag alone is 26kD.
Clone:: 2C4
Format:: Supplied as a liquid in PBS, pH 7.2. Labeled with Allophycocyanin (APC).
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. [provided by RefSeq Applications: Suitable for use in Immunofluorescence. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. AA Sequence: MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLMouse monoclonal antibody raised against a full length recombinant GCH1.YSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS Storage and Stability: Store product at 4°C in the dark. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Note: Applications are based on unconjugated antibody.

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