Figure 1
From: Predisposition of genetic disease by modestly decreased expression of GCH1 mutant allele

Metabolism of BH4. GTP is used for the synthesis of BH4. In the process of the synthesis of BH4, GTP cyclohydrolase is the first and the rate-limiting step enzyme. DHB or BH2, dihydrobiopterin; BH4, tetrahydrobiopterin.