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Product Description
GCH1 monoclonal Antibody | MB63388 | Bioworld
Host: Mouse
Reactivity: Human, Mouse, Rat
Application: IHC, WB
Application Range: WB 1:2000, IHC 1:500
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]
Storage & Stability: PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Specificity: GTP cyclohydrolase 1
Molecular Weight: 27.7 kDa (Predicted)
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B
Immunogen: Full length human recombinant protein of human GCH1 (NP_001019195) produced in E.coli.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
Pathway: N/A
