GCHFR binds to GCH1 and negatively regulates its activity

Stable Identifier
Reaction [binding]
Homo sapiens
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High levels of the end product, BH4, negatively regulates GCH1. It does this via GTP cyclohydrolase 1 feedback regulatory protein (GCHFR). BH4-dependant GCHFR in the form of a homopentamer complexes with the decameric GCH1 enzyme in the ratio 2:1 to inactivate it. L-phenylalanine reverses this inhibition. These regulatory steps control the biosynthesis of BH4. (Swick & Kapatos 2006, Chavan et al. 2006, Harada et al. 1993).

Literature References
PubMed ID Title Journal Year
16778797 GTP cyclohydrolase feedback regulatory protein controls cofactor 6-tetrahydrobiopterin synthesis in the cytosol and in the nucleus of epidermal keratinocytes and melanocytes

Gillbro, JM, Schallreuter, KU, Rokos, H, Chavan, B

J Invest Dermatol 2006
8502995 Feedback regulation mechanisms for the control of GTP cyclohydrolase I activity

Kagamiyama, H, Hatakeyama, K, Harada, T

Science 1993
16696853 A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions

Swick, L, Kapatos, G

J Neurochem 2006
Orthologous Events
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