Application
Western blot analysis of GCH-I expression in non-transfected: (A) and mouse GCH-I transfected: (B) 293T whole cell lysates.| Product Name | GCH-I Antibody (FL-250) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 1-250 representing full length GCH-I of human origin |
| Modification Notes | GTP cyclohydrolase I (GCH-I), a homodecamer, catalyzes the conversion of GTP into dihydroneopterin triphosphate and folate. GCH-I is the the first and rate limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis. BH4 is the cofactor for tyrosine hydroxylase, a rate-limiting enzyme for dopamine synthesis, and tryptophan hydroxylase, the rate-limiting enzyme for serotonin biosynthesis. Dopamine and serotonin are neurotransmitters involved in depression, which may be associated with a deficiency of BH4. Mutations in the gene encoding GCH-I cause malignant hyperphenylalaninemia, a genetic neurological disorder characterized by abnormally high levels of serum phenylalanine, and dopa-responsive dystonia (DRD), a group of movement disorders characterized by a progressive difficulty in walking which respond to L-dopa. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of GCH-I isoforms GCH-1, GCH-2, GCH-3 and GCH-4 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine and bovine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 1-250 representing full length GCH-I of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 1-250 representing full length GCH-I of human origin |
| Entrez Gene | 2643 |
|---|
Application
Western blot analysis of GCH-I expression in non-transfected: (A) and mouse GCH-I transfected: (B) 293T whole cell lysates.| Application Notes | recommended for detection of GCH-I isoforms GCH-1, GCH-2, GCH-3 and GCH-4 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine and bovine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
