Results 71 to 80 of about 4,605 (211)

Tetrahydrobiopterin Deficiency: From Phenotype to Genotype

Pteridines, 1993
As a result of the selective screening worldwide during the last 18 years, approximately 250 patients with tetrahydrobiopterin deficiency were discovered.
Blau Nenad, Thöny Beat, Heizmann Claus W., Dhondt Jean-Louis   +3 more
doaj   +1 more source

Advanced Research on Dopamine Signaling to Develop Drugs for the Treatment of Mental Disorders: Regulation of Dopaminergic Neural Transmission by Tyrosine Hydroxylase Protein at Nerve Terminals

Journal of Pharmacological Sciences, 2010
5R-l-Erythro-5,6,7,8-tetrahydrobiopterin (BH4) is an essential cofactor for tyrosine hydroxylase (TH). Recently, a type of dopa-responsive dystonia (DRD) (DYT5, Segawa’s disease) was revealed to be caused by dominant mutations of the gene encoding GTP ...
Chiho Sumi-Ichinose, Hiroshi Ichinose, Kazuhisa Ikemoto, Takahide Nomura, Kazunao Kondo   +4 more
doaj   +1 more source

Age‐Dependent Variation in Blood Biopterin Peaks Following Oral Tetrahydrobiopterin Administration in Phenylketonuria

Journal of Inherited Metabolic Disease, Volume 49, Issue 2, March 2026.
ABSTRACT The correct diagnosis of tetrahydrobiopterin (BH4, sapropterin dihydrochloride)‐responsive phenylketonuria (PKU) and treatment with BH4 are important for prognosis and quality of life. We examined whether age affects biopterin bioavailability following oral BH4 administration in PKU and whether this influences BH4 responsiveness.
Kana Kitayama, Tomoko Sakaguchi, Noriko Nakano, Daijiro Kabata, Haruo Shintaku, Takashi Hamazaki   +5 more
wiley   +1 more source

Stimulation of Tetrahydrobiopterin Synthesis by Cyclosporin A during Lipopolysaccharide Treatment in Vascular Endothelial Cells

Pteridines, 2002
We examined the effect of immunosuppressant cyclosporin A (CsA) on the synthesis of tetrahydrobiopterin (BH4), which is a cofactor for nitric oxide synthase (NOS), during treatment with lipopolysaccharide (LPS) in mouse brain microvascular endothelial ...
Ishii Masakazu, Shimizu Shunichi, Shiota Kazuhiro, Yamamoto Shinichiro, Kiuchi Yuji, Yamamoto Toshinori   +5 more
doaj   +1 more source

Identification of proteins interacting with GTP cyclohydrolase I

The FASEB Journal, 2008
GTP cyclohydrolase I (GCH‐1) is the rate limiting enzyme for biosynthesis of tetrahydrobiopterin (BH4) that is an essential cofactor for Nitric Oxide Synthase and aromatic amino acid hydroxylase. To explore the cell biology of GCH‐1 and determine the proteins interacting with GCH‐1, we cloned rat GCH‐1 linked with FLAG and established a stable HEK293 ...
Jianhai Du, Hao Xu, Na Wei, Basam Wakim, Kirkwood A Pritchard, Yang Shi   +5 more
openaire   +1 more source

RNA‐Binding Proteins and Ferroptosis in Cancer: Mechanism and Therapeutic Implications

MedComm – Oncology, Volume 5, Issue 1, March 2026.
Ferroptosis critically influences cancer cell fate and represents a promising therapeutic strategy. Emerging evidence identifies RNA‐binding proteins (RBPs) as key post‐transcriptional regulators of ferroptosis. The figure summarizes ferroptosis‐related RBPs across cancers: blue RBPs act as tumor suppressors by promoting ferroptosis, whereas red RBPs ...
Linlin Chang, Bin Lu, Haotian Shang, Wanjing Ding, Chunhua Wei, Xiaotian Qi, Pengxing He   +6 more
wiley   +1 more source

Melanisation of teladorsagia circumcincta larvae exposed to sunlight: a role for GTP-Cyclohydrolase in nematode survival

, 2012
Pre-parasitic stage larvae of a number of parasitic nematodes express high levels of the enzyme GTP-Cyclohydrolase I (GTP-CH). C. elegans mutants which are GTP-CH deficient have low levels of cuticualr melanin and show increased sensitivity to ...
Baker, R.H., Matthews, J.B., Nisbet, A.J., Britton, C., Roberts, B., Loer, C.M.   +5 more
core   +1 more source

Hippo Pathway Drives Durable Non‐Cell‐Autonomous Ferroptosis Resistance in Lung Cancer

Cancer Science, Volume 117, Issue 3, Page 683-694, March 2026.
YAP/TAZ, key effectors in lung cancer progression, paradoxically sensitize tumor cells to ferroptosis. This study reveals that YAP/TAZ‐low cells confer non‐cell‐autonomous, GCH1‐mediated durable ferroptosis resistance to neighboring YAP/TAZ‐high cells via a soluble factor, highlighting a Hippo pathway‐linked mechanism of tumor adaptation and survival ...
Mohamed Fathi Saleh, Akihiro Nita, Yudai Ohta, Aya Kobayashi, Hao Li, Yasuhisa Sakamoto, Toshiro Moroishi   +6 more
wiley   +1 more source

Flavin Biosynthesis Enhances Extracellular Electron Transfer in Bioengineered Escherichia coli

Advanced Science, Volume 13, Issue 2, 9 January 2026.
Microbial electronics are promising for energy, sensing, environmental, and synthesis applications. E. coli are engineered with extracellular electron transfer (EET) pathways from a microbe that naturally produces current to enable bioelectronics based on E. coli.
Mohammed Mouhib, Melania Reggente, Hanxuan Wang, Charlotte Roullier, Ardemis A. Boghossian   +4 more
wiley   +1 more source

Characterization of an Fe²⁺-Dependent Archaeal-Specific GTP Cyclohydrolase, MptA, from Methanocaldococcus jannaschii^†

, 2016
The first step in the biosynthesis of pterins in bacteria and plants is the conversion of GTP to 7,8-dihydro-d-neopterin triphosphate catalyzed by GTP cyclohydrolase I (GTPCHI).
Laura L. Grochowski (2489584), Robert H. White (1290399), Kapo Leung (166640), Huimin Xu (464647)   +3 more
core   +1 more source