Invitrogen
Catalog # PA5-50508
Quantity:
TESTED APPLICATIONS | DILUTION |
---|---|
Immunohistochemistry (Paraffin) (IHC (P)) | 1:5-1:20 |
Western Blot (WB) | 1:200-1:1,000 |
Species reactivity | Human |
Host / Isotype | Rabbit / IgG |
Class | Polyclonal |
Type | Antibody |
Immunogen | Synthetic peptide corresponding to a region derived from internal residues of human GTP cyclohydrolase 1 |
Conjugate | Unconjugated |
Form | Liquid |
Concentration | 0.3 mg/mL |
Purification | Antigen affinity chromatography |
Storage buffer | PBS, pH 7.4, with 40% glycerol |
Contains | 0.05% sodium azide |
Storage conditions | -20°C |
RRID | AB_2635961 |
The antibody detects endogenous levels of total GCH1 protein.
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.
Protein Aliases: dystonia 14; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I; guanosine 5'-triphosphate cyclohydrolase I
Gene Aliases: DYT14; DYT5; DYT5a; GCH; GCH1; GTP-CH-1; GTPCH1; HPABH4B
UniProt ID: (Human) P30793
Entrez Gene ID: (Human) 2643
Molecular Function:
hydrolase
metabolite interconversion enzyme
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