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|Tested species reactivity||Human|
|Host / Isotype||Goat / IgG|
|Immunogen||Synthetic peptide sequence (GKVHIGYLPNKQ) corresponding to the internal amino acids of GCH1|
|Purification||Antigen affinity chromatography|
|Storage buffer||TBS, pH 7.3, with 0.5% BSA|
|Contains||0.02% sodium azide|
|Storage Conditions||-20° C, Avoid Freeze/Thaw Cycles|
|Tested Applications||Dilution *|
|Western Blot (WB)||1-3 ug/ml|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
This antibody is predicted to react with mouse and rat based on sequence homology.
This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
dystonia 14; DYT5; GCH; GCH1; GTP cyclohydrolase 1 (dopa-responsive dystonia); GTP cyclohydrolase I; GTP-CH-1; GTP-CH-I; GTPCH1; guanosine 5'-triphosphate cyclohydrolase I
DYT14; DYT5; DYT5a; GCH; GCH1; GTP-CH-1; GTPCH1; HPABH4B