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GCH1 Antibody
SPA-04353
| 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. | 2C4 |
| Isotype | IgG1 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. |
For research use only. Not intended for any clinical use. No products from CD BioSciences may be resold, modified for resale or used to manufacture commercial products without prior written approval from CD BioSciences.