GTP cyclohydrolase I

GTP cyclohydrolase I

GTP cyclohydrolase I (GTP 7,8-8,9-dihydrolase, EC number|3.5.4.16) is an enzyme that is part of the folate and biopterin biosynthesis pathways. It is responsible for the hydrolysis of guanosine triphosphate (GTP) to form 7,8-dihydroneopterin 3'-triphosphate.

PBB_Summary
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summary_text = 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.cite web | title = Entrez Gene: GCH1 GTP cyclohydrolase 1 (dopa-responsive dystonia)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2643| accessdate = ]

References

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* [http://www.chem.qmul.ac.uk/iubmb/enzyme/EC3/5/4/16.html EC 3.5.4.16] IUBMB Enzyme Nomenclature

External links

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