Sulfa Drugs Inhibit Sepiapterin Reduction and Chemical Redox Cycling by Sepiapterin Reductase
نویسندگان
چکیده
منابع مشابه
Sulfa drugs inhibit sepiapterin reduction and chemical redox cycling by sepiapterin reductase.
Sepiapterin reductase (SPR) catalyzes the reduction of sepiapterin to dihydrobiopterin (BH2), the precursor for tetrahydrobiopterin (BH4), a cofactor critical for nitric oxide biosynthesis and alkylglycerol and aromatic amino acid metabolism. SPR also mediates chemical redox cycling, catalyzing one-electron reduction of redox-active chemicals, including quinones and bipyridinium herbicides (e.g...
متن کاملTwo Greek siblings with sepiapterin reductase deficiency.
BACKGROUND Sepiapterin reductase (SR) deficiency is a rare inherited disorder of neurotransmitter metabolism; less than 25 cases have been described in the literature so far. METHODS We describe the clinical history and extensive cerebrospinal fluid (CSF) and urine examination of two Greek siblings with the diagnosis of SR deficiency. The diagnosis was confirmed by enzyme activity measurement...
متن کاملDetection of sepiapterin in CSF of patients with sepiapterin reductase deficiency.
Sepiapterin reductase (SR) deficiency was recently described in patients with a severe biogenic amine deficiency presenting without hyperphenylalaninemia and it was suggested that the tetrahydrobiopterin (BH(4)) pathway may be different in different cells and tissues. We now developed a HPLC method for the measurement of yellow fluorescing sepiapterin for the rapid diagnosis of SR deficiency. S...
متن کاملSepiapterin reductase deficiency: two Indian siblings with unusual clinical features.
clinical symptoms of all MC were in line with classical PD. For comparison of AAO data, we used published AAO of a large population-based cohort. The AAO of 197 idiopathic PD patients was 70 6 11 years, whereas the AAO of MC with one mutation in our meta-analysis was 42 6 16 years (P < 0.0001). It should be noted however, patients with young onset of PD are more likely to be included in genetic...
متن کاملSepiapterin reductase regulation of endothelial tetrahydrobiopterin and nitric oxide bioavailability.
Sepiapterin reductase (SPR) catalyzes the final step of tetrahydrobiopterin (H(4)B) biosynthesis and the first step of H(4)B regeneration from an exogenous precursor sepiapterin. Despite the potential significance of SPR in regulating H(4)B-dependent nitric oxide (NO(*)) production, the endothelium-specific sequence and functions of SPR remain elusive. We first cloned endothelial SPR cDNA from ...
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ژورنال
عنوان ژورنال: Journal of Pharmacology and Experimental Therapeutics
سال: 2014
ISSN: 0022-3565,1521-0103
DOI: 10.1124/jpet.114.221572