The protein partners of GTP cyclohydrolase I in rat organs. [PDF]
PLoS ONE, 2012 GTP cyclohydrolase I (GCH1) is the rate-limiting enzyme for tetrahydrobiopterin biosynthesis and has been shown to be a promising therapeutic target in ischemic heart disease, hypertension, atherosclerosis and diabetes.
Jianhai Du +6 more
doaj +6 more sources
Atomic structure of GTP cyclohydrolase I [PDF]
Structure, 1995 Tetrahydrobiopterin serves as the cofactor for enzymes involved in neurotransmitter biosynthesis and as regulatory factor in immune cell proliferation and the biosynthesis of melanin. The biosynthetic pathway to tetrahydrobiopterin consists of three steps starting from GTP.
Herbert Nar +2 more
exaly +4 more sources
Induction of GTP cyclohydrolase I by bacterial lipopolysaccharide in the rat [PDF]
FEBS Letters, 1993 A 2‐ to 3‐fold increase of GTP cyclohydrolase I (E.C. 3.5.4.16), the key enzyme of tetrahydrobiopterin biosynthesis from GTP, was observed in cerebellum, remaining brain, liver, spleen, and adrenal gland of rats treated with a single dose of lipopolysaccharide (LPS). This led to increased biopterin levels in tissues but not in plasma.
Gabriele Werner-Felmayer +2 more
exaly +4 more sources
Elevated GTP Cyclohydrolase I Pathway in Endothelial Progenitor Cells of Overweight Premenopausal Women [PDF]
Cardiology Research and Practice, 2020 Background/Aims. Sexual differences exist in endothelial progenitor cells (EPCs), and various cardiovascular risk factors are associated with the preservation of endothelial function in premenopausal women.
Shaohong Wu +9 more
doaj +2 more sources
Inhibition of Brain GTP Cyclohydrolase I Attenuates 3-Nitropropionic Acid-Induced Striatal Toxicity: Involvement of Mas Receptor/PI3k/Akt/CREB/ BDNF Axis [PDF]
Frontiers in Pharmacology, 2021 GTP cyclohydrolase I (GTPCH I) is the rate-limiting enzyme for tetrahydrobiopterin (BH4) biosynthesis; the latter is an essential factor for iNOS activation that contributes neuronal loss in Huntington’s disease (HD).
Aya M. Mustafa +3 more
doaj +2 more sources
GTP-Cyclohydrolase I deficiency presenting as malignant hyperphenylalaninemia, recurrent hyperthermia and progressive neurological dysfunction in a South Asian child – a case report [PDF]
BMC Pediatrics, 2019 Background Tetrahydrobiopterin (BH4) deficiencies are disorders affecting phenylalanine homeostasis, and catecholamine and serotonin biosynthesis. GTP-Cyclohydrolase I deficiency (OMIM 600225) is an extremely rare variant of inborn error of BH4 synthesis
Kavinda Chandimal Dayasiri +6 more
doaj +2 more sources
Identification of proteins interacting with GTP cyclohydrolase I. [PDF]
Biochem Biophys Res Commun, 2009 GTP cyclohydrolase I (GCH-1) is the rate-limiting enzyme in the biosynthesis of tetrahydrobiopterin, an essential cofactor for nitric oxide synthase and aromatic amino acid hydroxylase. To explore the interactome of GCH-1, we established a HEK 293 cell line stably expressing tetracycline-inducible FLAG-GCH-1.
Du J +7 more
europepmc +4 more sources
Ligand binding to the inhibitory and stimulatory GTP cyclohydrolase I/GTP cyclohydrolase I feedback regulatory protein complexes [PDF]
Protein Science, 2001 AbstractGTP cyclohydrolase I feedback regulatory protein (GFRP) mediates feedback inhibition of GTP cyclohydrolase I activity by 6R‐l‐erythro‐5,6,7,8‐tetrahydrobiopterin (BH4), which is an essential cofactor for key enzymes producing catecholamines, serotonin, and nitric oxide as well as phenylalanine hydroxylase.
Kazuyuki Hatakeyama, Toshie Yoneyama
exaly +3 more sources
A hybrid approach reveals the allosteric regulation of GTP cyclohydrolase I. [PDF]
Proc Natl Acad Sci U S A, 2020 Significance We present a comprehensive structural study, which shows the human GCH1 and GCH1−GFRP complexes in all states (apo, ligand bound, partially and fully inhibited). We observed local rearrangements in the allosteric pocket upon BH4 binding, which result in drastic changes in the quaternary structure of the enzyme leading to a more ...
Ebenhoch R +11 more
europepmc +5 more sources
GTP cyclohydrolase I and tyrosine hydroxylase gene mutations in familial and sporadic dopa-responsive dystonia patients. [PDF]
PLoS ONE, 2013 Dopa-responsive dystonia (DRD) is a rare inherited dystonia that responds very well to levodopa treatment. Genetic mutations of GTP cyclohydrolase I (GCH1) or tyrosine hydroxylase (TH) are disease-causing mutations in DRD.
Chunyou Cai +8 more
doaj +2 more sources