Product Datasheet  
GCH1 Antibody  
Catalog Number: 36919  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#: P30793
    NCBI Gene ID: 2643
    Gene Accssion: NP_000152
  • Form of Antibody：  
  • Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Synthetic peptide corresponding to a region derived from internal residues of human GTP cyclohydrolase 1
  • reactivity：  
  • Hu
  • appl_detail：  
  • Western blotting: 1:200-1:1000
    Immunohistochemistry: 1:5-1:20
  • other_names：  
  • GCH; DYT5; DYT14; DYT5a; GTPCH1; HPABH4B; GTP-CH-1
  • Purification：  
  • Antigen affinity purification.
  • Specificity：  
  • The antibody detects endogenous levels of total GCH1 protein.
  • Applications：  
  • WB IHC
  • 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.



 
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