GCH1
Reactivity: Human
WB, ELISA
Host: Mouse
Polyclonal
unconjugated
Application Notes
Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: WB 1:500-2000,ELISA 1:5000-20000
Restrictions
For Research Use only
Format
Liquid
Concentration
1 mg/mL
Buffer
PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 %BSA
Preservative
ProClin
Precaution of Use
This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage
-20 °C
Storage Comment
Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.
Expiry Date
12 months
Target
GCH1
(GTP Cyclohydrolase 1 (GCH1))
Alternative Name
GCH1
Background
GTP cyclohydrolase 1, GTP cyclohydrolase I, GTP-CH-IGCH1 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. GCH1 (GTP Cyclohydrolase 1) is a Protein Coding gene. Diseases associated with GCH1 include Hyperphenylalaninemia, Bh4-Deficient, B and Dystonia, Dopa-Responsive, With Or Without Hyperphenylalaninemia. Among its related pathways are eNOS activation and regulation and Metabolism.