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https://www.readbyqxmd.com/read/28012135/eicosapentaenoic-acid-enriched-phosphatidylcholine-attenuated-hepatic-steatosis-through-regulation-of-cholesterol-metabolism-in-rats-with-nonalcoholic-fatty-liver-disease
#1
Yanjun Liu, Di Shi, Yingying Tian, Yuntao Liu, Qiping Zhan, Jie Xu, Jingfeng Wang, Changhu Xue
Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in the world. Disturbed cholesterol metabolism plays a crucial role in the development of NAFLD. The present study was conducted to evaluate the effects of EPA-PC extracted from sea cucumber on liver steatosis and cholesterol metabolism in NAFLD. Male Wistar rats were randomly divided into seven groups (normal control group, model group, lovastatin group, low- and high-dose EPA groups, and low- and high-dose EPA-PC groups). Model rats were established by administering a diet containing 1% orotic acid...
December 24, 2016: Lipids
https://www.readbyqxmd.com/read/28011993/putative-orotate-transporter-of-cryptococcus-neoformans-oat1-is-a-member-of-the-ncs1-prt-transporter-super-family-and-its-loss-causes-attenuation-of-virulence
#2
Akio Toh-E, Misako Ohkusu, Kiminori Shimizu, Azusa Takahashi-Nakaguchi, Susumu Kawamoto, Naruhiko Ishiwada, Akira Watanabe, Katsuhiko Kamei
It is well known that 5-fluoroorotic acid (5-FOA)-resistant mutants isolated from wild-type Cryptococcus neoformans are exclusively either ura3 or ura5 mutants. Unexpectedly, many of the 5-FOA-resistant mutants isolated in our selective regime were Ura(+). We identified CNM00460 as the gene responsible for these mutations. Cnm00460 belongs to the nucleobase cation symporter 1/purine-related transporter (NCS1/PRT) super family of fungal transporters, representative members of which are uracil transporter, uridine transporter and allantoin transporter of Saccharomyces cerevisiae...
December 23, 2016: Current Genetics
https://www.readbyqxmd.com/read/27928679/development-of-a-pyre-based-selective-system-for-thermotoga-sp-strain-rq7
#3
Dongmei Han, Zhaohui Xu
To fully unlock the biotechnological potentials of Thermotoga species, this study aimed to expand the genetic toolbox of Thermotoga by developing a new selective system. The developed system was composed of two components: a recipient strain bearing a deletion in its orotate phosphoribosyltransferase gene pyrE and a shuttle vector expressing a heterologous pyrE as the selectable marker. A spontaneous uracil auxotroph, T. sp. strain RQ7-15, was isolated at 70 °C with 2 mg/ml 5-fluoroorotic acid. The mutant carried a 112 bp deletion in pyrE and was a suitable recipient strain...
December 7, 2016: Extremophiles: Life Under Extreme Conditions
https://www.readbyqxmd.com/read/27906623/orotate-orotic-acid-an-essential-and-versatile-molecule
#4
M Löffler, E A Carrey, E Zameitat
Orotate (OA) is well-known as a precursor in biosynthesis of pyrimidines; in mammals it is released from the mitochondrial dihydroorotate dehydrogenase (DHODH) for conversion to UMP by the cytoplasmic UMP synthase enzyme. OA is also a normal part of the diet, being found in milk and dairy products, and it is converted to uridine for use in the pyrimidine salvage pathway predominantly in liver, kidney and erythrocytes. Early research into nutrition identified orotate as "vitamin B13," and its use as a complex with organic cations or metal ions was promulgated in body-building, and in assisting therapies of metabolic syndromes...
December 2016: Nucleosides, Nucleotides & Nucleic Acids
https://www.readbyqxmd.com/read/27574833/hereditary-orotic-aciduria-and-the-excretion-of-orotidine
#5
William L Nyhan, Jon A Gangoiti
Objective Orotic aciduria and deficiency of uridine monophosphate synthetase have been observed in a patient, studied over 10 years, who had no megaloblastic anemia. Excretion of orotic acid and orotidine were 8.24 and 0.52 mmol/mol of creatinine. The ratio of 15.85 differed appreciably from that of 6 patients reported with no megaloblastic anemia. Methods The analysis of orotidine by gas chromotography mass spectrometry was conducted. Conclusion Patients with orotic aciduria with and without megaloblastic anemia cannot be distinguished by ratio of orotic acid to orotidine...
December 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27370710/elevated-plasma-dihydroorotate-in-miller-syndrome-biochemical-diagnostic-and-clinical-implications-and-treatment-with-uridine
#6
John A Duley, Michael G Henman, Kevin H Carpenter, Michael J Bamshad, George A Marshall, Chee Y Ooi, Bridget Wilcken, Jason R Pinner
BACKGROUND: Miller syndrome (post-axial acrofacial dysostosis) arises from gene mutations for the mitochondrial enzyme dihydroorotate dehydrogenase (DHODH). Nonetheless, despite demonstrated loss of enzyme activity dihydroorotate (DHO) has not been shown to accumulate, but paradoxically urine orotate has been reported to be raised, confusing the metabolic diagnosis. METHODS: We analysed plasma and urine from a 4-year-old male Miller syndrome patient. DHODH mutations were determined by PCR and Sanger sequencing...
September 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27271695/metabolite-amyloids-a-new-paradigm-for-inborn-error-of-metabolism-disorders
#7
Ehud Gazit
The formation of ordered amyloid assemblies is associated with major human degenerative disorders, including Alzheimer's disease, Parkinson's disease, and type 2 diabetes. Amyloid fibrils are elongated nanoscale structures that bind to specific dyes (including thioflavin T and Congo red). Amyloid fibrillar assemblies or their early intermediates are known to induce apoptotic cytotoxic effect. Until recently, amyloid fiber formation was observed only with proteins and peptides. We reported in 2012 that a single amino acid, phenylalanine, could also form typical amyloid fibrils with the same morphology, dye-binding specificity, and electron diffraction pattern as protein amyloids...
July 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27147233/hyperammonemia-due-to-adult-onset-n-acetylglutamate-synthase-deficiency
#8
Anne-Els van de Logt, Leo A J Kluijtmans, Marleen C D G Huigen, Mirian C H Janssen
A 59-year-old woman, with a medical history of intellectual disability after perinatal asphyxia, was admitted because of coma due to hyperammonemia after she was treated for a fracture of the pelvis. The ammonia level was 280 μM. Acquired disorders as explanation for the hyperammonemia were excluded. Metabolic investigations showed an elevated glutamine and alanine and low citrulline, suspect for a urea cycle defect (UCD). Orotic acid could not be demonstrated in urine. DNA investigations were negative for mutations or deletions in the OTC and CPS1 gene, but revealed a homozygous c...
May 5, 2016: JIMD Reports
https://www.readbyqxmd.com/read/26995155/fetal-gene-therapy-for-ornithine-transcarbamylase-deficiency-by-intrahepatic-plasmid-dna-micro-bubble-injection-combined-with-hepatic-ultrasound-insonation
#9
Yoshie Oishi, Takashi Kakimoto, Wenji Yuan, Shuichi Kuno, Hiromasa Yamashita, Toshio Chiba
We evaluated the therapeutic efficacy of hepatic transfection of plasmid DNA using micro-bubbles and ultrasound insonation for fetal correction of ornithine transcarbamylase (OTC) deficiency in mice. Twenty-three sparse-fur heterozygous pregnant mice (day 16 of gestation) were divided into three groups: injection of plasmid-DNA micro-bubble mixture into fetal liver with ultrasound insonation (Tr, n = 11); control group 1 (C1), injection of plasmid-DNA micro-bubble mixture into fetal liver with no insonation (n = 5); and control group 2 (C2), injection of saline-micro-bubble mixture into fetal liver with ultrasound insonation (n = 7)...
June 2016: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/26861612/investigation-of-vital-pathogenic-target-orotate-phosphoribosyltransferases-oprtase-from-thermus-thermophilus-hb8-phylogenetic-and-molecular-modeling-approach
#10
Kanagarajan Surekha, Damodharan Prabhu, Mariadasse Richard, Mutharasappan Nachiappan, Jayashree Biswal, Jeyaraman Jeyakanthan
Biosynthesis pathways of pyrimidine and purine are shown to play an important role in regular cellular activities. The biosynthesis can occur either through de novo or salvage pathways based on the requirement of the cell. The pyrimidine biosynthesis pathway has been linked to several disorders and various autoimmune diseases. Orotate phosphoribosyl transferase (OPRTase) is an important enzyme which catalyzes the conversion of orotate to orotate monophosphate in the fifth step of pyrimidine biosynthesis. Phylogenetic analysis of 228 OPRTase sequences shows the distribution of proteins across different living forms of life...
June 1, 2016: Gene
https://www.readbyqxmd.com/read/26775542/long-term-fatty-liver-induced-insulin-resistance-in-orotic-acid-induced-nonalcoholic-fatty-liver-rats
#11
Xiuqing Han, Chunhua Liu, Yong Xue, Jingfeng Wang, Changhu Xue, Teruyoshi Yanagita, Xiang Gao, Yuming Wang
We investigated whether fatty liver preceded insulin resistance or vice versa using a long-term orotic acid (OA)-induced nonalcoholic fatty liver disease (NAFLD) model without the confounding effects of obesity and hyperlipidemia and explored the role of the liver in insulin resistance. Male Wistar rats were fed with or without OA supplementation for 30, 60, and 90 days. The NAFLD group showed increased liver lipid at 30, 60, and 90 days; glucose intolerance was noted at 60 and 90 days. Furthermore, partial liver proteins and gene expressions related to upstream signaling of insulin were decreased...
2016: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/26726740/ability-of-food-drink-to-reduce-the-bitterness-intensity-of-topiramate-as-determined-by-taste-sensor-analysis
#12
Tamami Haraguchi, Takahiro Uchida, Mai Hazekawa, Miyako Yoshida, Masaki Nakashima, Hotaka Sanda, Takema Hase, Yutaka Tomoda
The purpose of this study was to determine which foods and/or drinks are capable of reducing the bitterness of topiramate when consumed together with the medicine. The inhibitory effects of foods/drinks (yoghurt and nine other foods/drinks) on the bitterness of topiramate (5 mg/mL) were evaluated with a taste sensor using a bitterness-responsive membrane (C00). The effect of topiramate on the taste characteristics of the foods/drinks themselves was also evaluated by taste sensor outputs. The viscosities of the foods/drinks and the influence of the lactic acid and orotic acid components of yoghurt, the most successful of the tested substances in taste masking, on the bitterness of topiramate were also measured...
2016: Chemical & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/26707627/production-of-orotic-acid-by-a-klura3%C3%AE-mutant-of-kluyveromyces-lactis
#13
Nuno Carvalho, Eduardo Coelho, Luís Gales, Vítor Costa, José António Teixeira, Pedro Moradas-Ferreira
We demonstrated that a Klura3Δ, mutant of the yeast Kluyveromyces lactis is able to produce and secrete into the growth medium considerable amounts of orotic acid. Using yeast extract-peptone-glucose (YPD) based media we optimized production conditions in flask and bioreactor cultures. With cells grown in YPD 5% glucose medium, the best production in flask was obtained with a 1:12.5 ratio for flask: culture volume, 180 rpm, 28°C and 200 mM MOPS for pH stabilization at neutral values (initial culture pH at 8...
June 2016: Journal of Bioscience and Bioengineering
https://www.readbyqxmd.com/read/26639842/effects-of-fatty-liver-induced-by-excess-orotic-acid-on-b-group-vitamin-concentrations-of-liver-blood-and-urine-in-rats
#14
Katsumi Shibata, Nobuya Morita, Tomoyo Kawamura, Ai Tsuji, Tsutomu Fukuwatari
Fatty liver is caused when rats are given orotic acid of the pyrimidine base in large quantities. The lack of B-group vitamins suppresses the biosynthesis of fatty acids. We investigated how orotic acid-induced fatty liver affects the concentrations of liver, blood, and urine B-group vitamins in rats. The vitamin B6 and B12 concentrations of liver, blood, and urine were not affected by orotic acid-induced fatty liver. Vitamin B2 was measured only in the urine, but was unchanged. The liver, blood, and urine concentrations of niacin and its metabolites fell dramatically...
2015: Journal of Nutritional Science and Vitaminology
https://www.readbyqxmd.com/read/26604374/the-effect-of-terebinth-pistacia-terebinthus-l-coffee-addition-on-the-chemical-and-physical-characteristics-colour-values-organic-acid-profiles-mineral-compositions-and-sensory-properties-of-ice-creams
#15
Arzu Kavaz Yüksel, Ihsan Güngör Şat, Mehmet Yüksel
The aim of this research was to evaluate the effect of terebinth (Pistacia terebinthus L.) coffee addition (0.5, 1 and 2 %) on the chemical and physical properties, colour values, organic acid profiles, mineral contents and sensory characteristics of ice creams. The total solids, fat, titratable acidity, viscosity, first dripping time and complete melting time values, a (*) and b (*) colour properties, citric, lactic, acetic and butyric acid levels and Ca, Cu, Mg, Fe, K, Zn and Na concentrations of ice creams showed an increase with the increment of terebinth coffee amount, while protein, pH, L (*), propionic acid and orotic acid values decreased...
December 2015: Journal of Food Science and Technology
https://www.readbyqxmd.com/read/26446336/a-novel-mutation-in-ornithine-transcarbamylase-gene-causing-mild-intermittent-hyperammonemia
#16
Sarar Mohamed, Muddathir H Hamad, Altaf A Kondkar, Khaled K Abu-Amero
We report a 3-year-old Saudi boy with recurrent episodes of vomiting, poor feeding, and altered mental status accompanied by an intermittent mild hyperammonemia, and a large elevation of urinary orotic acid. Sanger sequencing of the ornithine transcarbamylase (OTC) gene revealed a novel hemizygous deletion at the fourth nucleotide of intron 4 (c.386+4delT) in the proband and his asymptomatic mother. This novel mutation in the OTC gene is responsible for the late-onset phenotype of OTC deficiency.
October 2015: Saudi Medical Journal
https://www.readbyqxmd.com/read/26304391/metabolomics-and-microbiological-profile-of-italian-mozzarella-cheese-produced-with-buffalo-and-cow-milk
#17
Maria Barbara Pisano, Paola Scano, Antonio Murgia, Sofia Cosentino, Pierluigi Caboni
Italian buffalo mozzarella (BM) cheese metabolite profile and microbial communities were characterised and compared to cow mozzarella (CM). Polar metabolite profiles were studied by gas-chromatography mass-spectrometry (GC-MS) and results elaborated by multivariate analysis (MVA). BM produced using natural whey starter cultures (NWS) exhibited a higher microbial diversity with less psychrotrophic bacteria. BM samples were higher in threonine, serine, valine, and lower in orotic acid and urea. CM produced with commercial starters (CMS) had the highest count of Streptococcus thermophilus and higher levels of galactose and phenylalanine...
February 1, 2016: Food Chemistry
https://www.readbyqxmd.com/read/26080667/-report-of-a-case-with-late-onset-ornithine-transcarbamylase-deficiency-with-gas-chromatography-mass-spectrometry-and-dna-sequencing-confirmation-and-literatures-review
#18
Wenjia Tong, Danqun Jin, Jingmin Sun
OBJECTIVE: To discuss the clinical characteristics, diagnosis and treatment management of ornithine transcarbamylase deficiency (OTCD). METHOD: Data of the clinical diagnosis and treatment of a case with OTCD were analyzed, and the domestic and international literature was reviewed. RESULT: (1) The case was a boy, 8 years old; and the initial symptoms were vomiting and reduced consciousness for a day after eating a lot of eggs as previous similar history...
May 2015: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/26059769/orotic-acid-more-than-just-an-intermediate-of-pyrimidine-de-novo-synthesis
#19
REVIEW
Monika Löffler, Elizabeth A Carrey, Elke Zameitat
It is timely to consider the many facets of the small molecule orotic acid (OA), which is well-known as an essential intermediate of pyrimidine de novo synthesis. In addition, it can be taken up by erythrocytes and hepatocytes for conversion into uridine and for use in the pyrimidine recycling pathway. We discuss the link between dietary orotate and fatty liver in rats, and the potential for the alleviation of neonatal hyperbilirubinaemia. We address the development of orotate derivatives for application as anti-pyrimidine drugs, and of complexes with metal ions and organic cations to assist therapies of metabolic syndromes...
May 20, 2015: Journal of Genetics and Genomics, Yi Chuan Xue Bao
https://www.readbyqxmd.com/read/26026930/sensitive-and-selective-determination-of-orotic-acid-in-biological-specimens-using-a-novel-fluorogenic-reaction
#20
Sheng Yin, Shpend Dragusha, Valon Ejupi, Takayuki Shibata, Tsutomu Kabashima, Masaaki Kai
Orotic acid is an intermediate in the synthesis pathway of uridine-5'-monophosphate, and increases in body fluids of patients suffering from hereditary disorders such as orotic aciduria and hyperammonemia. In this study, we developed a spectrofluorometric method with or without high-performance liquid chromatography for the selective and sensitive quantification of orotic acid in human biological specimens, using 4-trifluoromethylbenzamidoxime (4-TFMBAO) as a fluorogenic reagent. This reagent provided intensive fluorescence for only orotic acid amongst 62 compounds including structurally related bio-substances such as nucleic acid bases, nucleosides, nucleotides, amino acids, vitamins, bilirubin, uric acid, urea, creatine, creatinine and sugars...
July 2015: Journal of Fluorescence
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