Disorders of biopterin metabolism [PDF]
Journal of Inherited Metabolic Disease, 2009 SummaryDefects in the metabolism or regeneration of tetrahydrobiopterin (BH4) were initially discovered in patients with hyperphenylalaninaemia who had progressive neurological deterioration despite optimal metabolic control (malignant hyperphenylalaninaemia).
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A unique dual activity amino acid hydroxylase in Toxoplasma gondii [PDF]
, 2009 The genome of the protozoan parasite Toxoplasma gondii was found to contain two genes encoding tyrosine hydroxylase; that produces L-DOPA. The encoded enzymes metabolize phenylalanine as well as tyrosine with substrate preference for tyrosine.
Gaskell, EA +4 more
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Tetrahydro‐6‐biopterin is associated with tetrahydro‐7‐biopterin in primary murine mast cells [PDF]
FEBS Letters, 1992 Murine bone marrow‐derived mast cells proliferate in response to interleukin 3. In addition to 6‐biopterin, 7‐biopterin was identified in these cells by HPLC analysis of iodine oxidized extracts and by alkaline permanganate oxidation to the 6‐ and 7‐carboxylic acids. 7‐Biopterin comprised 31.9 (± 7.7)% of the total biopterin.
Ziegler, Irmgard, Hültner, Lothar
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Isolation and culture of murine bone marrow-derived macrophages for nitric oxide and redox biology [PDF]
, 2020 Macrophages are mononuclear phagocytes derived from haematopoietic progenitors that are widely distributed throughout the body. These cells participate in both innate and adaptive immune responses and lie central to the processes of inflammation ...
Andrew Shaw +37 more
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Inflammation and oxidative stress caused by nitric oxide synthase uncoupling might lead to left ventricular diastolic and systolic dysfunction in patients with hypertension [PDF]
, 2015 Objective: To investigate the role of oxidative stress, inflammation, hypercoagulability and neuroendocrine activation in the transition of hypertensive heart disease to heart failure with preserved ejection fraction (HFPEF).
Bodó I +14 more
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Nitric oxide and hydrogen sulfide: the gasotransmitter paradigm of the vascular system. [PDF]
, 2017 There are several reviews on NO and hydrogen sulfide (H2 S) and their role in vascular diseases in the current relevant literature. The aim of this review is to discuss, within the limits of present knowledge, the interconnection between these two ...
Bucci, M., Cirino, G, Vellecco, V
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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
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Biopterin in Parkinson's disease. [PDF]
Journal of Neurology, Neurosurgery & Psychiatry, 1987 Tetrahydrobiopterin is an essential co-factor in the natural synthesis of dopamine. Oral tetrahydrobiopterin was given in small doses to four patients with early Parkinson's disease but had no discernible effect.
A P, Moore +3 more
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Effects of endothelin-1 on intracellular tetrahydrobiopterin levels in vascular tissue [PDF]
, 2018 Objective: Tetrahydrobiopterin (BH4) is the essential cofactor of endothelial nitric oxide synthase (eNOS) and intracellular levels of BH4 is regulated by oxidative stress.
Antoniades, Charalambos +6 more
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Inherited metabolic epilepsies–established diseases, new approaches
Epilepsia Open, EarlyView.Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
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