GCHFR explained

GTP cyclohydrolase 1 feedback regulatory protein is an enzyme that in humans is encoded by the GCHFR gene.^{[1] [2] [3]}

GTP cyclohydrolase I feedback regulatory protein binds to and mediates tetrahydrobiopterin inhibition of GTP cyclohydrolase I. The regulatory protein, GCHFR, consists of a homodimer. It is postulated that GCHFR may play a role in regulating phenylalanine metabolism in the liver and in the production of biogenic amine neurotransmitters and nitric oxide.

Further reading

• Maruyama K, Sugano S . Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides . Gene . 138 . 1–2 . 171–4 . 1994 . 8125298 . 10.1016/0378-1119(94)90802-8 .
• Yoneyama T, Brewer JM, Hatakeyama K . GTP cyclohydrolase I feedback regulatory protein is a pentamer of identical subunits. Purification, cDNA cloning, and bacterial expression . J. Biol. Chem. . 272 . 15 . 9690–6 . 1997 . 9092499 . 10.1074/jbc.272.15.9690 . free .
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K . Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library . Gene . 200 . 1–2 . 149–56 . 1997 . 9373149 . 10.1016/S0378-1119(97)00411-3 . etal.
• Bader G, Schiffmann S, Herrmann A . Crystal structure of rat GTP cyclohydrolase I feedback regulatory protein, GFRP . J. Mol. Biol. . 312 . 5 . 1051–7 . 2001 . 11580249 . 10.1006/jmbi.2001.5011 . etal.
• Werner ER, Bahrami S, Heller R, Werner-Felmayer G . Bacterial lipopolysaccharide down-regulates expression of GTP cyclohydrolase I feedback regulatory protein . J. Biol. Chem. . 277 . 12 . 10129–33 . 2002 . 11799107 . 10.1074/jbc.M107326200 . free .
• Strausberg RL, Feingold EA, Grouse LH . Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences . Proc. Natl. Acad. Sci. U.S.A. . 99 . 26 . 16899–903 . 2003 . 12477932 . 10.1073/pnas.242603899 . 139241 . 2002PNAS...9916899M . etal. free .
• Gesierich A, Niroomand F, Tiefenbacher CP . Role of human GTP cyclohydrolase I and its regulatory protein in tetrahydrobiopterin metabolism . Basic Res. Cardiol. . 98 . 2 . 69–75 . 2003 . 12607127 . 10.1007/s00395-003-0394-y . 19864675 .
• Gerhard DS, Wagner L, Feingold EA . The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC) . Genome Res. . 14 . 10B . 2121–7 . 2004 . 15489334 . 10.1101/gr.2596504 . 528928 . etal.
• Kalivendi S, Hatakeyama K, Whitsett J . Changes in tetrahydrobiopterin levels in endothelial cells and adult cardiomyocytes induced by LPS and hydrogen peroxide--a role for GFRP? . Free Radic. Biol. Med. . 38 . 4 . 481–91 . 2006 . 15649650 . 10.1016/j.freeradbiomed.2004.11.004 . etal.
• . GTP cyclohydrolase feedback regulatory protein controls cofactor 6-tetrahydrobiopterin synthesis in the cytosol and in the nucleus of epidermal keratinocytes and melanocytes . J. Invest. Dermatol. . 126 . 11 . 2481–9 . 2007 . 16778797 . 10.1038/sj.jid.5700425 . free .
• Ohta E, Funayama M, Ichinose H . Novel mutations in the guanosine triphosphate cyclohydrolase 1 gene associated with DYT5 dystonia . Arch. Neurol. . 63 . 11 . 1605–10 . 2006 . 17101830 . 10.1001/archneur.63.11.1605 . etal.
• Kontostavlaki DP, Panayotacopoulou MT, Sluijs JA . Co-expression of tyrosine hydroxylase and GTP cyclohydrolase I in arginine vasopressin-synthesizing neurons of the human supraoptic nucleus demonstrated by laser microdissection and real-time PCR . Neuroendocrinology . 84 . 6 . 386–95 . 2007 . 17135716 . 10.1159/000097487 . 40550512 . etal.
• Nandi M, Kelly P, Vallance P, Leiper J . Over-expression of GTP-cyclohydrolase 1 feedback regulatory protein attenuates LPS and cytokine-stimulated nitric oxide production . Vasc. Med. . 13 . 1 . 29–36 . 2008 . 18372436 . 10.1177/1358863X07085916 . 8626710 .
• Ionova IA, Vasques-Vivar J, Whitsett J . Deficient BH4 production via de novo and salvage pathways regulates NO responses to cytokines in adult cardiac myocytes . Am. J. Physiol. . 295 . 5 . H2178–87 . 2008 . 18835915 . 10.1152/ajpheart.00748.2008 . 2614582 . etal.

Notes and References

1. Milstien S, Jaffe H, Kowlessur D, Bonner TI . Purification and cloning of the GTP cyclohydrolase I feedback regulatory protein, GFRP . J. Biol. Chem. . 271 . 33 . 19743–51 . Oct 1996 . 8702680 . 10.1074/jbc.271.33.19743 . free .
2. Hochstrasser DF, Frutiger S, Paquet N, Bairoch A, Ravier F, Pasquali C, Sanchez JC, Tissot JD, Bjellqvist B, Vargas R . Human liver protein map: a reference database established by microsequencing and gel comparison . Electrophoresis . 13 . 12 . 992–1001 . Mar 1993 . 1286669 . 10.1002/elps.11501301201 . 23518983 . etal.
3. Web site: Entrez Gene: GCHFR GTP cyclohydrolase I feedback regulator.