GTP cyclohydrolase 1 (GCH1) Rabbit Polyclonal Antibody

CAT#: TA321072

Anti-GCH1 Rabbit Polyclonal Antibody


USD 380.00

In Stock*

Size
    • 100 ul

Product Images

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Specifications

Product Data
Applications IHC, WB
Recommended Dilution ELISA: 1:1000-2000, WB: 1:200-1000, IHC: 1:5-20
Reactivities Human, Mouse, Rat
Host Rabbit
Isotype IgG
Clonality Polyclonal
Immunogen Synthetic peptide corresponding to a region derived from 51-64 amino acids of human GTP cyclohydrolase 1
Formulation PBS pH7.3, 0.05% NaN3, 50% glycerol
Concentration lot specific
Purification Antigen affinity purification
Conjugation Unconjugated
Storage Store at -20°C as received.
Stability Stable for 12 months from date of receipt.
Predicted Protein Size 28 kDa
Gene Name GTP cyclohydrolase 1
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.
Synonyms DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B
Reference Data
Protein Families Druggable Genome
Protein Pathways Folate biosynthesis, Metabolic pathways

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