Dystonia and Biopterin Deficiency
Five patients with childhood dystonia associated with reduced CSF biopterin, responsive to levodopa, and characterized by diurnal and exertional variation are reported from the Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, MD.
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Redox sensor and effector functions of tetrahydrobiopterin and the mitochondria in molecular models of cardiovascular disease [PDF]
Tetrahydrobiopterin (BH4) is a redox active molecule that is essential for the production of nitric oxide (NO) by the NO synthase (NOS) enzymes. Loss of endothelial BH4 leads to decreased NO and increased superoxide generation by endothelial NOS (eNOS ...
Bailey, Jade
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Long-term Western diet fed apolipoprotein E-deficient rats exhibit only modest early atherosclerotic characteristics [PDF]
In the apolipoprotein E–deficient mouse, the gut microbiota has an impact on the development of atherosclerosis, but whether such correlations are also present in rats requires investigation.
Bornfeldt, Karin E. +14 more
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Effects of peri-operative statin treatment on atrial electrical properties, post-operative atrial fibrillation and in-hospital clinical outcomes in patients undergoing elective cardiac surgery [PDF]
Surgical myocardial revascularization remains the standard of care for patients with multi-vessel coronary artery disease. A growing body of evidence indicates that systemic inflammation and myocardial oxidative stress are associated with the ...
Jayaram, Raja
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Quantification of Neurotransmitters in Mouse Brain Tissue by Using Liquid Chromatography Coupled Electrospray Tandem Mass Spectrometry [PDF]
A simple and rapid liquid chromatography tandem mass spectrometry method has been developed for the determination of BH4, DA, 5-HT, NE, EP, Glu, and GABA in mouse brain using epsilon-acetamidocaproic acid and isotopically labeled neurotransmitters as ...
Hak Rim Kim +3 more
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Roles for endothelial cell and macrophage Gch1 and tetrahydrobiopterin in atherosclerosis progression [PDF]
GTP cyclohydrolase I (GTPCH) catalyses the first and rate-limiting reaction in the synthesis of tetrahydrobiopterin (BH4), an essential cofactor for nitric oxide synthases (NOS).
Al Haj Zen, Ayman +9 more
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Pharmacological therapies for Machado-Joseph disease [PDF]
Machado-Joseph disease (MJD), also known as Spinocerebellar Ataxia type 3 (SCA3), is the most common autosomal dominant ataxia worldwide. MJD integrates a large group of disorders known as polyglutamine diseases (polyQ).
Maciel, P., Silva, Sara Carina Duarte
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Pteridines, sterols, and indole derivatives from the lithistid sponge Corallistes undulatus of the Coral Sea [PDF]
D'Ambrosio, M. +4 more
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Nitric oxide, superoxide, and peroxynitrite: Synthesis, interactions, and biological consequences. [PDF]
The control of nitric oxide (NO) production and its interaction with other free radicals were examined in vitro and in vivo. First, the role of calmodulin in the regulation of inducible NO synthase (iNOS) was assessed.
Amirmansour, Charles
core
ADCY5-related movement disorders: Frequency, disease course and phenotypic variability in a cohort of paediatric patients [PDF]
Bhatia, KP +19 more
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