GCH1 monoclonal Antibody | MB63388

GCH1 monoclonal Antibody | MB63388 | Gentaur UK, US & Europe Distribution

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