Reactome | GCHFR binds to GCH1 and negatively regulates its activity

GCHFR binds to GCH1 and negatively regulates its activity

Stable Identifier

R-HSA-1474158

Type

Reaction [binding]

Species

Homo sapiens

Compartment

cytosol

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GCHFR binds to GCH1 and negatively regulates its activity

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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
8502995	Feedback regulation mechanisms for the control of GTP cyclohydrolase I activity Harada, T, Kagamiyama, H, Hatakeyama, K	Science	1993
16778797	GTP cyclohydrolase feedback regulatory protein controls cofactor 6-tetrahydrobiopterin synthesis in the cytosol and in the nucleus of epidermal keratinocytes and melanocytes Chavan, B, Gillbro, JM, Rokos, H, Schallreuter, KU	J Invest Dermatol	2006
16696853	A yeast 2-hybrid analysis of human GTP cyclohydrolase I protein interactions Swick, L, Kapatos, G	J Neurochem	2006