GTP cyclohydrolase 1 (GCH1) Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI5A1]
Other products for "GCH1"
Specifications
Product Data | |
Clone Name | OTI5A1 |
Applications | IHC, WB |
Recommended Dilution | WB 1:2000, IHC 1:500 |
Reactivities | Human, Mouse, Rat |
Host | Mouse |
Isotype | IgG1 |
Clonality | Monoclonal |
Immunogen | Full length human recombinant protein of human GCH1 (NP_001019195) produced in E.coli. |
Formulation | PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide. |
Concentration | 0.5 mg/ml |
Purification | Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G) |
Conjugation | Biotin |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 27.7 kDa |
Gene Name | GTP cyclohydrolase 1 |
Database Link | |
Background | 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, Jul 2008] |
Synonyms | DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Folate biosynthesis, Metabolic pathways |
Documents
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