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Authored: Jassal, B, 2011-08-17, Edited: Jassal, B, 2011-08-17, Reviewed: D'Eustachio, P, 2011-08-23, The first and rate-limiting enzyme in tetrahydrobiopterin de novo biosynthesis is GTP cyclohydrolase I (GCH1, GTPCHI). Three different isoforms are produced but only isoform 1 is functionally active (Gütlich et al. 1994). GCH1 is functional as a homodecamer. First, a monomer of GCH1 forms a dimer. Then five dimers arrange into a ring-like structure to form the homodecamer (Nar et al. 1995).
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GCH1 reduces GTP to dihydroneopterin triphosphate
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3.5.4.16
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