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 +5 more
wiley +1 more source
Increasing Tetrahydrobiopterin in Cardiomyocytes Adversely Affects Cardiac Redox State and Mitochondrial Function Independently of Changes in NO Production [PDF]
, 2016 Tetrahydrobiopterin (BH4) represents a potential strategy for the treatment of cardiac remodeling, fibrosis and/or diastolic dysfunction. The effects of oral treatment with BH4 (Sapropterin™ or Kuvan™) are however dose-limiting with high dose negating ...
Bennett, Brian +5 more
core +1 more source
Radical-mediated ring contraction in the biosynthesis of 7-deazapurines [PDF]
, 2015 Pyrrolopyrimidine containing natural products are widely distributed in Nature. The biosynthesis of the 7-deazapurine moiety that is common to all pyrrolopyrimidines entails multiple steps, one of which is a complex radical-mediated ring contraction ...
Bandarian, Vahe, Drennan, Catherine L.
core +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 +6 more
wiley +1 more source
Primary Disorders of Neurotransmitter Metabolism: Experience of a Tertiary Center [PDF]
, 2011 Neurotransmitter diseases are a group of inherited disorders attributable to a disturbance of neurotransmitter metabolism. Biogenic amines are neurotransmitters with multiple roles including psychomotor function, hormone secretion, cardiovascular ...
Calado, E +3 more
core
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 +6 more
wiley +1 more source
The International Working Group on Neurotransmitter related Disorders (iNTD): A worldwide research project focused on primary and secondary neurotransmitter disorders [PDF]
, 2016 INTRODUCTION: Neurotransmitters are chemical messengers that enable communication between the neurons in the synaptic cleft. Inborn errors of neurotransmitter biosynthesis, breakdown and transport are a group of very rare neurometabolic diseases ...
Cortès-Saladelafont, Elisenda +17 more
core +4 more sources
Mutationen im PTS-Gen und mögliche Auswirkungen auf Funktion und Struktur der 6-Pyruvoyl-Tetrahydropterin-Synthase [PDF]
, 2002 Background: Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of patients with JIA.
Preuße, Friedrich-Alexander +1 more
core +9 more sources
Functional Studies on the Promoter Region of the Human GTP Cyclohydrolase I Gene
Pteridines, 1995 We have isolated genomic clones that contained the gene for human GTP cyclohydrolase I. One clone containing the 5'-regulatory region of this gene was further analysed.
Gütlich Markus +6 more
doaj +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 +4 more
wiley +1 more source