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GCH1, Mouse, Clone: 1F5, Abnova™
Mouse monoclonal antibody raised against a full-length recombinant GCH1.
Supplier: Abnova Corporation H00002643M03
Description
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
Sequence: MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRSSpecifications
GCH1 | |
Monoclonal | |
Unconjugated | |
PBS with no preservative; pH 7.4 | |
BC025415 | |
GCH1 | |
GCH1 (AAH25415.1, 1 a.a. ∼ 250 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. | |
100 μg | |
Primary | |
Human | |
Antibody | |
IgG2b κ |
ELISA, Immunofluorescence | |
1F5 | |
Mouse monoclonal antibody raised against a full length recombinant GCH1. | |
GCH1 | |
DYT14/DYT5/GCH/GTP-CH-1/GTPCH1 | |
Mouse | |
Affinity chromatography | |
RUO | |
2643 | |
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing. | |
Liquid |
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