Results 61 to 70 of about 5,611 (205)

A Putative Guanosine Triphosphate Cyclohydrolase I Named CaGCH1 Is Involved in Hyphal Branching and Fruiting Development in Cyclocybe aegerita

Frontiers in Microbiology, 2022
Guanosine triphosphate (GTP) cyclohydrolase I (GCH1) is the limiting enzyme of the tetrahydrobiopterin (BH4) synthesis pathway. The disruption of gch1 gene may cause conditional lethality due to folic acid auxotrophy in microorganisms, although the ...
Nan Tao, Nan Tao, Nan Tao, Bopu Cheng, Bopu Cheng, Hongmei Chai, Hongmei Chai, Hongmei Chai, Xianghua Cui, Xianghua Cui, Xianghua Cui, Yuanhao Ma, Yuanhao Ma, Yuanhao Ma, Jinping Yan, Yongchang Zhao, Yongchang Zhao, Yongchang Zhao, Weimin Chen, Weimin Chen, Weimin Chen   +20 more
doaj   +1 more source

Vitamin‐Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy

Journal of Inherited Metabolic Disease, Volume 49, Issue 4, July 2026.
ABSTRACT Vitamin‐dependent cofactors are essential for numerous metabolic reactions, and defects affecting their uptake, conversion, utilisation, or regeneration constitute a heterogeneous group of inherited metabolic disorders (IMDs). Although dietary vitamin intake is sufficient to sustain coenzyme synthesis in healthy individuals, it is insufficient
Cécile Acquaviva, Yann Nadjar, Manuel Schiff, Christine Vianey‐Saban, Alain Fouilhoux, Jean‐François Benoist   +5 more
wiley   +1 more source

Decameric GTP Cyclohydrolase I Forms Complexes with Two Pentameric GTP Cyclohydrolase I Feedback Regulatory Proteins in the Presence of Phenylalanine or of a Combination of Tetrahydrobiopterin and GTP [PDF]

Journal of Biological Chemistry, 1998
The activity of GTP cyclohydrolase I is inhibited by (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin (BH4) and stimulated by phenylalanine through complex formation with GTP cyclohydrolase I feedback regulatory protein (GFRP). Gel filtration experiments as well as enzyme activity measurements showed that the number of subunits of GFRP in both the inhibitory
T, Yoneyama, K, Hatakeyama
openaire   +2 more sources

GTP cyclohydrolase I expression, protein, and activity determine intracellular tetrahydrobiopterin levels, independent of GTP cyclohydrolase feedback regulatory protein expression. [PDF]

, 2009
GTP cyclohydrolase I (GTPCH) is a key enzyme in the synthesis of tetrahydrobiopterin (BH4), a required cofactor for nitricoxide synthases and aromatic amino acid hydroxylases.
Channon, Keith, Warrick, N, Crabtree, MJ, Alp, NJ, Channon, KM, Tatham, AL, Cai, S   +6 more
core   +1 more source

Cardiovascular Benefits of Salvia miltiorrhiza (Danshen) Supplementation: Bioactive Constituents, Mechanisms, Clinical Evidence and Implications for Nutritional Care

Food Science &Nutrition, Volume 14, Issue 5, May 2026.
Salvia miltiorrhiza (Danshen) contains bioactive tanshinones, including tanshinone IIA, cryptotanshinone, and tanshinone I, that exert cardioprotective effects through coordinated modulation of vascular endothelial growth factor receptor (VEGFR), transforming growth factor beta 1 (TGF‐β1), transforming growth factor beta receptor (TGF‐βR), toll‐like ...
Akash Vikal, Rashmi Maurya, Preeti Patel, Balak Das Kurmi, William N. Setzer, Javad Sharifi‐Rad, Daniela Calina   +6 more
wiley   +1 more source

Statin Increases GTP Cyclohydrolase I mRNA and 5,6,7,8-Tetrahydrobiopterin in Vascular Endothelial Cells

Pteridines, 2006
We investigated the effects of HMG-CoA reductase inhibitors, so-called statins, on 5,6,7,8-tctrahydrobioptcrin (BH4) metabolism in human umbilical vein endothelial cells (HUVEC).
Hattori Yoshiyuki, Nakanishi Nobuo
doaj   +1 more source

Lack of influence of GTP cyclohydrolase gene (GCH1) variations on pain sensitivity in humans

Molecular Pain, 2007
Objectives To assess the effect of variations in GTP cyclohydrolase gene (GCH1) on pain sensitivity in humans. Methods Thermal and cold pain sensitivity were evaluated in a cohort of 735 healthy volunteers.
Dionne Raymond A, Kim Hyungsuk
doaj   +1 more source

A Novel Multimodal LC–MS/MS Panel for the Comprehensive Diagnosis of Neurometabolic Disorders in CSF

Journal of Inherited Metabolic Disease, Volume 49, Issue 3, May 2026.
ABSTRACT Metabolic testing of cerebrospinal fluid (CSF) is essential for early diagnosis of neurometabolic disorders. However, the large number of differential diagnoses, the phenotypic variance within a clinical picture, and the disease rarity complicate targeted metabolic diagnostics.
Stine Christ, Julia Rossmann, Sylvia Richter, Sven Garbade, Glynis Klinke, Nenad Blau, Georg Friedrich Hoffmann, Jürgen Günther Okun, Thomas Opladen   +8 more
wiley   +1 more source

GTP cyclohydrolase I utilizes metal-free GTP as its substrate [PDF]

, 2004
GTP cyclohydrolase I (GCH) is the rate-limiting enzyme for the synthesis of tetrahydrobiopterin and its activity is important in the regulation of monoamine neurotransmitters such as dopamine, norepinephrine and serotonin.
Hiroshi Ichinose, Takahiro Suzuki, Hideki Kurita   +2 more
core  

Genetic diagnosis of two dopa-responsive dystonia families by exome sequencing. [PDF]

PLoS ONE, 2014
Dopa-responsive dystonia, a rare disorder typically presenting in early childhood with lower limb dystonia and gait abnormality, responds well to levodopa. However, it is often misdiagnosed with the wide spectrum of phenotypes.
Zhan-fang Sun, Yu-han Zhang, Ji-feng Guo, Qi-ying Sun, Jun-pu Mei, Han-lin Zhou, Li-ping Guan, Jin-yong Tian, Zheng-mao Hu, Jia-da Li, Kun Xia, Xin-xiang Yan, Bei-sha Tang   +12 more
doaj   +1 more source