GCH1 Antibody - CD BioSciences

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GCH1 Antibody

GCH1 Antibody

SPA-04354

Size Price
0.1 mL Online Inquiry
25 µL Online Inquiry
More Options Online Inquiry
Target Information
Target Name GCH1
Gene Abbr. GCH1
Gene ID 2643
Full Name GTP cyclohydrolase 1
Alias DYT14, DYT5, DYT5a, GCH, GTP-CH-1
Introduction 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]
Product Details
Host Mouse
Clonality Monoclonal
Clone No. 1F5
Isotype IgG2B Kappa
Immunogen 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. MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS.
Usage
Application ELISA, IF
Reactivity Human
Specificity GCH1 - GTP cyclohydrolase 1 (dopa-responsive dystonia).
Storage & Handling
Storage Buffer In 1X PBS, pH 7.4.
Preservative No Preservative
Storage Temp. Aliquot and store at -20 °C or -80 °C.
Handling Avoid freeze-thaw cycles.

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