GCHFR

GTP cyclohydrolase 1 feedback regulatory protein is an enzyme that in humans is encoded by the GCHFR gene.[5][6][7]

GCHFR
Identifiers
Aliases	GCHFR, GFRP, HsT16933, P35, GTP cyclohydrolase I feedback regulator
External IDs	OMIM: 602437 MGI: 2443977 HomoloGene: 3849 GeneCards: GCHFR
Gene location (Human) Chr. Chromosome 15 (human)[1] Band 15q15.1 Start 40,764,068 bp[1] End 40,767,708 bp[1]
Gene location (Mouse) Chr. Chromosome 2 (mouse)[2] Band 2|2 E5 Start 119,167,773 bp[2] End 119,172,390 bp[2]
RNA expression pattern More reference expression data
Gene ontology Molecular function • GTP cyclohydrolase binding • GO:0048551 enzyme inhibitor activity • amino acid binding • GO:0001948 protein binding • enzyme binding • GTP-dependent protein binding Cellular component • cytoplasm • cytosol • nuclear membrane • membrane • melanosome • dendrite • cell nucleus • nucleoplasm • macromolecular complex Biological process • nitric oxide biosynthetic process • protein heterooligomerization • negative regulation of GTP cyclohydrolase I activity • neurotransmitter metabolic process • negative regulation of biosynthetic process • macromolecular complex assembly Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	2644	320415
Ensembl	ENSG00000137880	ENSMUSG00000046814
UniProt	P30047	P99025
RefSeq (mRNA)	NM_005258	NM_177157
RefSeq (protein)	NP_005249	NP_796131
Location (UCSC)	Chr 15: 40.76 – 40.77 Mb	Chr 2: 119.17 – 119.17 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

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.[7]

References

1. GRCh38: Ensembl release 89: ENSG00000137880 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000046814 - Ensembl, May 2017
3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
5. Milstien S, Jaffe H, Kowlessur D, Bonner TI (Oct 1996). "Purification and cloning of the GTP cyclohydrolase I feedback regulatory protein, GFRP". J. Biol. Chem. 271 (33): 19743–51. doi:10.1074/jbc.271.33.19743. PMID 8702680.
6. Hochstrasser DF, Frutiger S, Paquet N, Bairoch A, Ravier F, Pasquali C, Sanchez JC, Tissot JD, Bjellqvist B, Vargas R, et al. (Mar 1993). "Human liver protein map: a reference database established by microsequencing and gel comparison". Electrophoresis. 13 (12): 992–1001. doi:10.1002/elps.11501301201. PMID 1286669.
7. "Entrez Gene: GCHFR GTP cyclohydrolase I feedback regulator".

Further reading

• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
• Yoneyama T, Brewer JM, Hatakeyama K (1997). "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. doi:10.1074/jbc.272.15.9690. PMID 9092499.
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
• Bader G, Schiffmann S, Herrmann A, et al. (2001). "Crystal structure of rat GTP cyclohydrolase I feedback regulatory protein, GFRP". J. Mol. Biol. 312 (5): 1051–7. doi:10.1006/jmbi.2001.5011. PMID 11580249.
• Werner ER, Bahrami S, Heller R, Werner-Felmayer G (2002). "Bacterial lipopolysaccharide down-regulates expression of GTP cyclohydrolase I feedback regulatory protein". J. Biol. Chem. 277 (12): 10129–33. doi:10.1074/jbc.M107326200. PMID 11799107.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "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. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Gesierich A, Niroomand F, Tiefenbacher CP (2003). "Role of human GTP cyclohydrolase I and its regulatory protein in tetrahydrobiopterin metabolism". Basic Res. Cardiol. 98 (2): 69–75. doi:10.1007/s00395-003-0394-y. PMID 12607127.
• Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
• Kalivendi S, Hatakeyama K, Whitsett J, et al. (2006). "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. doi:10.1016/j.freeradbiomed.2004.11.004. PMID 15649650.
• Chavan B, Gillbro JM, Rokos H, Schallreuter KU (2007). "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. doi:10.1038/sj.jid.5700425. PMID 16778797.
• Ohta E, Funayama M, Ichinose H, et al. (2006). "Novel mutations in the guanosine triphosphate cyclohydrolase 1 gene associated with DYT5 dystonia". Arch. Neurol. 63 (11): 1605–10. doi:10.1001/archneur.63.11.1605. PMID 17101830.
• Kontostavlaki DP, Panayotacopoulou MT, Sluijs JA, et al. (2007). "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. doi:10.1159/000097487. PMID 17135716.
• Nandi M, Kelly P, Vallance P, Leiper J (2008). "Over-expression of GTP-cyclohydrolase 1 feedback regulatory protein attenuates LPS and cytokine-stimulated nitric oxide production". Vasc. Med. 13 (1): 29–36. doi:10.1177/1358863X07085916. PMID 18372436.
• Ionova IA, Vasques-Vivar J, Whitsett J, et al. (2008). "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. doi:10.1152/ajpheart.00748.2008. PMC 2614582. PMID 18835915.