Results 11 to 20 of about 5,611 (205)

GTP Cyclohydrolase II Structure and Mechanism [PDF]

Journal of Biological Chemistry, 2005
GTP cyclohydrolase II converts GTP to 2,5-diamino-6-beta-ribosyl-4(3H)-pyrimidinone 5'-phosphate, formate and pyrophosphate, the first step in riboflavin biosynthesis. The essential role of riboflavin in metabolism and the absence of GTP cyclohydrolase II in higher eukaryotes makes it a potential novel selective antimicrobial drug target.
Ren J, Kotaka M, Lockyer M, Lamb HK, Hawkins AR, Stammers DK   +5 more
openaire   +7 more sources

Reduction of GTP Cyclohydrolase I Feedback Regulating Protein Expression by Hydrogen Peroxide in Vascular Endothelial Cells [PDF]

Journal of Pharmacological Sciences, 2005
We examined the effect of H2O2 on the expression of GTP cyclohydrolase I (GTPCH) feedback regulating protein (GFRP). Addition of H2O2 to endothelial cells decreased GFRP mRNA levels, in contrast to the increase of tetrahydrobiopterin (BH4) content and ...
Masakazu Ishii, Shunichi Shimizu, Teruaki Wajima, Tamio Hagiwara, Takaharu Negoro, Akira Miyazaki, Takashi Tobe, Yuji Kiuchi   +7 more
doaj   +2 more sources

Pulmonary Hypertension in a GTP-Cyclohydrolase 1–Deficient Mouse [PDF]

Circulation, 2005
Background— GTP-cyclohydrolase 1 (GTP-CH1) catalyzes the first step for the de novo production of tetrahydrobiopterin (BH 4 ), a cofactor for nitric oxide synthase (NOS). The hyperphenylalaninemic mutant mouse (hph-1) displays a 90% reduction in GTP-CH1 activity. Reduced BH
Nandi, M, Miller, A, Stidwill, R, Jacques, TS, Lam, AA, Haworth, S, Heales, S, Vallance, P   +7 more
openaire   +4 more sources

Generation of an iPSC line from a patient with GTP cyclohydrolase 1 (GCH1) deficiency: HDMC0061i-GCH1 [PDF]

Stem Cell Research, 2017
Fibroblasts from a female patient carrying a heterozygous variation in GTP cyclohydrolase 1 (GCH1; OMIM: 600225; HGNC: 4193; c.235_240del/p.(L79_S80del)), the rate-limiting enzyme of tetrahydrobiopterin (BH4) synthesis, were reprogrammed to iPSCs using ...
Sabine Jung-Klawitter, Juliane Ebersold, Gudrun Göhring, Nenad Blau, Thomas Opladen   +4 more
doaj   +3 more sources

Mechanism and catalytic strategy of the prokaryotic-specific GTP cyclohydrolase-IB. [PDF]

Biochem J, 2017
GTP cyclohydrolase I catalyzes the first step in folic acid biosynthesis in bacteria and plants, biopterin biosynthesis in mammals, and the biosynthesis of 7-deazaguanosine modified tRNA nucleosides in bacteria and archaea. The type IB GTP cyclohydrolase
Paranagama N, Bonnett SA, Alvarez J, Luthra A, Stec B, Gustafson A, Iwata-Reuyl D, Swairjo MA.   +7 more
europepmc   +3 more sources

Parkinson's disease in GTP cyclohydrolase 1 mutation carriers. [PDF]

Brain, 2015
Sir, We read with great interest the study titled ‘Parkinson’s disease in GTP cyclohydrolase 1 mutation carriers’ in the September edition of Brain (Mencacci et al. , 2014). The study demonstrates loss-of-function variants in the GCH1 gene are not only a major cause of DOPA-responsive dystonia but are also enriched in relatives with adult-onset ...
Ryan BJ, Crabtree MJ, Channon KM, Wade-Martins R.   +3 more
europepmc   +7 more sources

Parkinsonism in GTP cyclohydrolase 1 mutation carriers. [PDF]

Brain, 2015
Sir, We read with great interest the article by Mencacci and colleagues (2014) reporting a significantly higher frequency of GTP cyclohydrolase 1 ( GCH1 ) variants in patients with Parkinson’s disease compared to controls. Whole-exome sequencing of a large case-control cohort showed that rare GCH1 coding variants are associated with a 7-fold increased
Guella I, Sherman HE, Appel-Cresswell S, Rajput A, Rajput AH, Farrer MJ.   +5 more
europepmc   +4 more sources

Structures and reaction mechanisms of GTP cyclohydrolases [PDF]

IUBMB Life, 2013
AbstractGTP cyclohydrolases generate the first committed intermediates for the biosynthesis of certain vitamins/cofactors (folic acid, riboflavin, deazaflavin, and tetrahydrobiopterin), deazapurine antibiotics, some t‐RNA bases (queuosine, archaeosine), and the phytotoxin, toxoflavin.
Tobias, Gräwert, Markus, Fischer, Adelbert, Bacher   +2 more
openaire   +2 more sources

Parkinson's disease in GTP cyclohydrolase 1 mutation carriers. [PDF]

Brain, 2014
GTP cyclohydrolase 1, encoded by the GCH1 gene, is an essential enzyme for dopamine production in nigrostriatal cells. Loss-of-function mutations in GCH1 result in severe reduction of dopamine synthesis in nigrostriatal cells and are the most common cause of DOPA-responsive dystonia, a rare disease that classically presents in childhood with ...
Mencacci NE, Isaias IU, Reich MM, Ganos C, Plagnol V, Polke JM, Bras J, Hersheson J, Stamelou M, Pittman AM, Noyce AJ, Mok KY, Opladen T, Kunstmann E, Hodecker S, Münchau A, Volkmann J, Samnick S, Sidle K, Nanji T, Sweeney MG, Houlden H, Batla A, Zecchinelli AL, Pezzoli G, Marotta G, Lees A, Alegria P, Krack P, Cormier-Dequaire F, Lesage S, Brice A, Heutink P, Gasser T, Lubbe SJ, Morris HR, Taba P, Koks S, Majounie E, Raphael Gibbs J, Singleton A, Hardy J, Klebe S, Bhatia KP, Wood NW, International Parkinson’s Disease Genomics Consortium and UCL-exomes consortium.   +45 more
europepmc   +7 more sources

GTP Cyclohydrolase 1/Tetrahydrobiopterin Counteract Ferroptosis through Lipid Remodeling [PDF]

, 2020
Ferroptosis is an iron-dependent form of regulated cell death linking iron, lipid, and glutathione levels to degenerative processes and tumor suppression.
Stefanie M. Hauck (295113), Joel A. Schick (8194404), Susanne Pfeiffer (4615381), Jacob D. Daniels (3145668), Brent R. Stockwell (30593), Juliane Merl-Pham (626353), Carla T. Bezjian (8194392), Larissa Ringelstetter (8194395), Johanna Kössl (8194401), Fereshteh Zandkarimi (1321719), Philippe Schmitt-Kopplin (82664), Natasa Anastasov (6166622), Vanessa A. N. Kraft (8194389), Stefanie Brandner (1998154), Constanze Müller (8194398), Xuanwen Bao (6630359), Kamyar Hadian (749171)   +16 more
core   +7 more sources