GTP cyclohydrolase 1 (GCH1) Rabbit Polyclonal Antibody
CNY 1,999.00
CNY 3,280.00
货期*
2周
规格
经常一起买 (1)
beta Actin Mouse Monoclonal Antibody, Clone OTI1, Loading Control
CNY 300.00
CNY 1,430.00
Specifications
Product Data | |
Applications | ICC/IF, WB |
Recommend Dilution | WB,1:500 - 1:2000 IF,1:50 - 1:200 |
Reactivity | Human, Mouse, Rat |
Modifications | Unmodified |
Host | Rabbit |
Clonality | Polyclonal |
Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human GCH1 (NP_000152.1). |
Formulation | Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |
Concentration | lot specific |
Purification | Affinity purification |
Conjugation | Unconjugated |
Storage Condition | Store at -20℃. Avoid freeze / thaw cycles. |
Predicted Protein Size | 23kDa/25kDa/27kDa |
Gene Name | GTP cyclohydrolase 1 |
Database Link | |
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; GTP-CH-I; GTPCH1; HPABH4B |
Reference Data |
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