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NH2-triP is then transformed into 6-pyruvoyltetrahydrobiopterin (6-PTP) by 6-pyruvoyl tetrahydrobiopterin synthase (PTPS), and finally, 6-PTP is reduced to BH4 via three successive reactions catalyzed by sepiapterin reductase (SPR)

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[DOI] [PubMed] [Google Scholar] 61.Wang Y.C., Wu P.H., Ting W.C., Wang Y.F., Yang M.H., Su T.H., Su J.Y., Sun H.I., Huang W.M., Tsai P.L., et al

doi: 10.1056/NEJM200507073530117 8

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