GCH1
GTP cyclohydrolase 1
Synonyms:
DYT14, DYT5, DYT5a, GCH, GTP-CH-1, GTPCH1, HPABH4B
Species:
Human GCH1 (2643), Mouse Gch1 (14528), Rat Gch1 (29244), Zebrafish gch1 (100192219), sheep GCH1 (101112987), domestic guinea pig Gch1 (100735198), naked mole-rat Gch1 (101706667), Domestic Rabbit GCH1 (100286831), cow GCH1 (286815), sheep GCH1 (100303455) ...More
UniProtKB ID:
Gene ID:
- Human(2643) Summary: 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. [provided by RefSeq, Jul 2008]
- Mouse(14528) GTP cyclohydrolase 1
- Rat(29244) GTP cyclohydrolase 1
- Zebrafish(100192219) GTP cyclohydrolase 1
- sheep(101112987) GTP cyclohydrolase 1
- domestic guinea pig(100735198) GTP cyclohydrolase 1
- naked mole-rat(101706667) GTP cyclohydrolase 1
- Domestic Rabbit(100286831) GTP cyclohydrolase 1
- cow(286815) GTP cyclohydrolase 1
- sheep(100303455) GTP cyclohydrolase 1
- dog(609393) GTP cyclohydrolase 1
- domestic cat(101092120) GTP cyclohydrolase 1
- chicken(396146) GTP cyclohydrolase 1
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Antibodies
Product Number
Description
Species Reactivity
Application
Monoclonal Anti-GTP cyclohydrolase I antibody produced in rat, ~1.0 mg/mL, clone MGTP 6H11, purified immunoglobulin,
Species Reactivity
human, rat
Application
immunocytochemistry
immunoprecipitation (IP)
western blot
immunoprecipitation (IP)
western blot
Anti-GCH1 antibody produced in goat, affinity isolated antibody, buffered aqueous solution,
Species Reactivity
human
Application
ELISA
immunohistochemistry
immunohistochemistry
Anti-GCH1 antibody produced in rabbit, purified immunoglobulin, buffered aqueous solution,
Species Reactivity
human
Application
western blot