GTP-CH-1 Antibody
Product Details
- Cat. No.
- 250680
- Type
- Primary Antibody
- Clonality
- Polyclonal
- Host
- Rabbit
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Description
GTP cyclohydrolase 1 (GTP-CH-1 or GCH-1) catalyzes the conversion of GTP to D-erythro-7,8-dihydroneopterin triphosphate, the first and rate-limiting step in tetrahydrobiopterin (BH4) biosynthesis. The final product (BH4) is an essential co-factor for three aromatic amino acid monooxygenases (F, Y, and W). Isoform GCH-1 is the functional enzyme. GTP-CH-1 positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVEC). GTP-CH-1 deficiency is a cause of malignant hyperphenylalaninemia due to tetrahydrobiopterin deficiency. It is also responsible for defective neurotransmission due to depletion of the neurotransmitters dopamine and serotonin. GTP-CH-1 (DYT5) is a cause for dystonia 5, a DOPA-responsive dystonia, defined by sustained involuntary muscle contractions.
Biological Information
- Clonality: Polyclonal
- Host: Rabbit
- Reactivity: Human, Mouse, Rat
- Antigen: KLH-conjugated synthetic peptide encompassing a sequence within the N-term region of human GTP-CH-1.
- Isotype: Rabbit Ig
- Gene: P30793
Handling
- Quantity: 0.1 mg
- Storage: Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Applications
- ELISA (ELISA)
- Immunohistochemistry (IHC)
- Western Blotting (WB)