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https://www.readbyqxmd.com/read/28526534/3-methylglutaconic-aciduria-a-frequent-but-underrecognized-finding-in-carbamoyl-phosphate-synthetase-i-deficiency
#1
Dariusz Rokicki, Magdalena Pajdowska, Joanna Trubicka, Meow-Keong Thong, Elżbieta Ciara, Dorota Piekutowska-Abramczuk, Maciej Pronicki, Roman Sikora, Rijad Haidar, Mariusz Ołtarzewski, Ewa Jabłońska, Premala Muthukumarasamy, Pavai Sthaneswar, Chin-Seng Gan, Małgorzata Krajewska-Walasek, Rosalba Carrozzo, Daniela Verrigni, Michela Semeraro, Cristiano Rizzo, Roberta Taurisano, Bader Alhaddad, Reka Kovacs-Nagy, Tobias B Haack, Carlo Dionisi-Vici, Ewa Pronicka, Saskia B Wortmann
The urea cycle disorder carbamoyl phosphate synthetase I deficiency is an important differential diagnosis in the encephalopathic neonate. This intoxication type inborn error of metabolism often leads to neonatal death or severe and irreversible damage of the central nervous system, even despite appropriate treatment. Timely diagnosis is crucial, but can be difficult on routine metabolite level. Here, we report ten neonates from eight families (finally) diagnosed with CPS1 deficiency at three tertiary metabolic centres...
May 16, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28526122/uptake-of-dissolved-inorganic-and-organic-nitrogen-by-the-benthic-toxic-dinoflagellate-ostreopsis-cf-ovata
#2
Cécile Jauzein, Douglas Couet, Thierry Blasco, Rodolphe Lemée
Environmental factors that shape dynamics of benthic toxic blooms are largely unknown. In particular, for the toxic dinoflagellate Ostreopsis cf. ovata, the importance of the availability of nutrients and the contribution of the inorganic and organic pools to growth need to be quantified in marine coastal environments. The present study aimed at characterizing N-uptake of dissolved inorganic and organic sources by O. cf. ovata cells, using the (15)N-labelling technique. Experiments were conducted taking into account potential interactions between nutrient uptake systems as well as variations with the diel cycle...
May 2017: Harmful Algae
https://www.readbyqxmd.com/read/28526121/rates-of-nitrogen-uptake-by-cyanobacterially-dominated-assemblages-in-lake-taihu-china-during-late-summer
#3
Jingjing Yang, Han Gao, Patricia M Glibert, Yu Wang, Mengmeng Tong
Lake Taihu has suffered an increasing number of cyanobacteria harmful algal blooms (CyanoHABs) over the past three decades, bringing about formidable ecological and economical losses. Efforts to control phosphate (P) and/or nitrogen (N) have been applied to mitigate these blooms, but there has been much less attention paid to N and its different forms on the ecology of the blooms. Therefore, kinetic and nutrient enrichment experiments were conducted to assess N uptake rates under differing conditions, and to examine effects of changes in N forms (NH4(+), NO3(-) and urea) and P availability on phytoplankton community physiology...
May 2017: Harmful Algae
https://www.readbyqxmd.com/read/28525556/transcriptome-of-the-gsh-depleted-lens-reveals-changes-in-detoxification-and-emt-signaling-genes-transport-systems-and-lipid-homeostasis
#4
Jeremy A Whitson, Xiang Zhang, Mario Medvedovic, Jenny Chen, Zongbo Wei, Vincent M Monnier, Xingjun Fan
Purpose: To understand the effects of glutathione (GSH)-deficiency on genetic processes that regulate lens homeostasis and prevent cataractogenesis. Methods: The transcriptome of lens epithelia and fiber cells was obtained from C57BL/6 LEGSKO (lens GSH-synthesis knockout) and buthionine sulfoximine (BSO)-treated LEGSKO mice and compared to C57BL/6 wild-type mice using RNA-Seq. Transcriptomic data were confirmed by qPCR and Western blot/ELISA on a subset of genes...
May 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28521975/facile-synthesis-of-cellulose-based-carbon-with-tunable-n-content-for-potential-supercapacitor-application
#5
Zehong Chen, Xinwen Peng, Xiaoting Zhang, Shuangshuang Jing, Linxin Zhong, Runcang Sun
Producing hierarchical porous N-doped carbon from renewable biomass is an essential and sustainable way for future electrochemical energy storage. Herein we cost-efficiently synthesized N-doped porous carbon from renewable cellulose by using urea as a low-cost N source, without any activation process. The as-prepared N-doped porous carbon (N-doped PC) had a hierarchical porous structure with abundant macropores, mesopores and micropores. The doping N resulted in more disordered structure, and the doping N content in N-doped PC could be easily tunable (0...
August 15, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/28500942/synthesis-of-high-surface-area-mesoporous-mno2-via-a-metastable-aqueous-interfacial-reaction
#6
Xingpu Wang, Shuhui Huo, Rongfang Wang, Hui Wang, Dan J L Brett, Shan Ji
In this work, a metastable aqueous interface was fabricated for synthesizing mesoporous and high surface area MnO2. When urea was used as the additive, hierarchical spheres self-organized from ultrathin nano-sheets were obtained. Its porous structure could be controlled through adjusting the urea concentration, and a maximum surface area of 407m(2) g(-1) was achieved by this method, which is larger than the reported values. Due to the porous structure and high surface area, as-prepared MnO2 exhibited a specific capacitance of 775...
May 4, 2017: Journal of Colloid and Interface Science
https://www.readbyqxmd.com/read/28498888/cardiomyocyte-specific-loss-of-mitochondrial-p32-c1qbp-causes-cardiomyopathy-and-activates-stress-responses
#7
Toshiro Saito, Takeshi Uchiumi, Mikako Yagi, Rie Amamoto, Daiki Setoyama, Yuichi Matsushima, Dongchon Kang
Aims: Mitochondria are important organelles, dedicated to energy production. Mitochondrial p32/C1qbp, which functions as an RNA and protein chaperone, interacts with mitochondrial mRNA and is indispensable for mitochondrial function through its regulation of mitochondrial translation in cultured cell lines. However, the precise role of p32/C1qbp in vivo is poorly understood because of embryonic lethality in the systemic p32-deficient mouse. The goal of this study was to examine the physiological function of mitochondrial p32/C1qbp in the heart...
May 11, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28498114/life-cycle-assessment-of-sludge-management-with-phosphorus-utilisation-and-improved-hygienisation-in-sweden
#8
M Svanström, S Heimersson, G Peters, R Harder, D I'Ons, A Finnson, J Olsson
To provide input to sewage sludge management strategies that address expected new regulations in terms of hygienisation and phosphorus recovery in Sweden, an environmental life cycle assessment (LCA) was made. The LCA identified environmental hot spots for methods that may permit sludge or phosphorus from sludge to be applied on agricultural land. In particular, thermophilic digestion, pasteurisation, thermal hydrolysis, urea treatment and mono-incineration with phosphorus recovery were compared. In addition, a sludge management system involving drying of sludge before use in forestry was investigated...
May 2017: Water Science and Technology: a Journal of the International Association on Water Pollution Research
https://www.readbyqxmd.com/read/28496435/serum-metabolomic-profiling-of-piglets-infected-with-virulent-classical-swine-fever-virus
#9
Wenjie Gong, Junjie Jia, Bikai Zhang, Shijiang Mi, Li Zhang, Xiaoming Xie, Huancheng Guo, Jishu Shi, Changchun Tu
Classical swine fever (CSF) is a highly contagious swine infectious disease and causes significant economic losses for the pig industry worldwide. The objective of this study was to determine whether small molecule metabolites contribute to the pathogenesis of CSF. Birefly, serum metabolomics of CSFV Shimen strain-infected piglets were analyzed by ultraperformance liquid chromatography/electrospray ionization time-of-flight mass spectrometry (UPLC/ESI-Q-TOF/MS) in combination with multivariate statistical analysis...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28492150/selective-screening-for-organic-acidurias-and-amino-acidopathies-in-pakistani-children
#10
Noreen Abbas Sherazi, Aysha Habib Khan, Lena Jafri, Azeema Jamil, Nasir Ali Khan, Bushra Afroze
OBJECTIVE: To determine the frequency of organic acidurais (OA) and amino acidopathies (AA) in selected high-risk patients screened in two years. STUDY DESIGN: Retrospective Observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital (AKUH), Karachi, from January 2013 to December 2014. METHODOLOGY: Patients with OA and AA were included in the study and patients with IMDs other than OA and AA were excluded...
April 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28481923/nitrapyrin-addition-mitigates-nitrous-oxide-emissions-and-raises-nitrogen-use-efficiency-in-plastic-film-mulched-drip-fertigated-cotton-field
#11
Tao Liu, Yongchao Liang, Guixin Chu
Nitrification inhibitors (NIs) have been used extensively to reduce nitrogen losses and increase crop nitrogen nutrition. However, information is still scant regarding the influence of NIs on nitrogen transformation, nitrous oxide (N2O) emission and nitrogen utilization in plastic-film-mulched calcareous soil under high frequency drip-fertigated condition. Therefore, a field trial was conducted to evaluate the effect of nitrapyrin (2-chloro-6-(trichloromethyl)-pyridine) on soil mineral nitrogen (N) transformation, N2O emission and nitrogen use efficiency (NUE) in a drip-fertigated cotton-growing calcareous field...
2017: PloS One
https://www.readbyqxmd.com/read/28470097/phenylbutyrate-and-%C3%AE-cell-function-contribution-of-histone-deacetylases-and-er-stress-inhibition
#12
Sabbir Khan, Sandeep K Komarya, Gopabandhu Jena
Incidences of diabetes are increasing globally due to involvement of genetic and epigenetic factors. Phenylbutyrate (PBA) is a US FDA approved drug for treatment of urea cycle disorder in children. PBA reduces endoplasmic reticulum (ER) stress and is proven as a potent histone deacetylases (HDACs) inhibitor. Chronic ER stress results in unfolding protein response, which triggers apoptosis. Abnormal ER homoeostasis is responsible for defective processing of several genes/proteins and contributes to β-cell death/failure...
May 4, 2017: Epigenomics
https://www.readbyqxmd.com/read/28462819/human-esc-ipsc-derived-hepatocyte-like-cells-achieve-zone-specific-hepatic-properties-by-modulation-of-wnt-signaling
#13
Seiji Mitani, Kazuo Takayama, Yasuhito Nagamoto, Kazuo Imagawa, Fuminori Sakurai, Masashi Tachibana, Ryo Sumazaki, Hiroyuki Mizuguchi
The function of hepatocytes largely depends on their position in the liver lobule. Although the method of differentiating hepatocytes from human pluripotent stem cells has been largely improved over the past decade, there remains no technique for generating hepatocyte-like cells (HLCs) with zone-specific hepatic properties. In this study, we searched for the factors that promote acquisition of zone-specific properties of HLCs. Here, we identified that WNT7B and WNT8B secreted from hepatocytes and cholangiocytes play important roles in achieving perivenous zone-specific characteristics, such as the enhancement of glutamine secretion, citric acid cycle, cytochrome P450 (CYP) 1A2 metabolism, and CYP1A2 induction capacities...
April 24, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28458255/sirt5-binds-to-cardiolipin-and-regulates-the-electron-transport-chain
#14
Yuxun Zhang, Sivakama S Bharathi, Matthew J Rardin, Jie Lu, Katherine V Maringer, Sunder Sims-Lucas, Edward V Prochownik, Bradford W Gibson, Eric S Goetzman
SIRT5 is a lysine desuccinylase known to regulate mitochondrial fatty acid oxidation and the urea cycle. Here, SIRT5 was observed to bind to cardiolipin via an amphipathic helix on its amino terminus. In vitro, succinyl-CoA was used to succinylate liver mitochondrial membrane proteins. SIRT5 largely reversed the succinyl-CoA-driven lysine succinylation. Quantitative mass spectrometry of SIRT5-treated membrane proteins pointed to the electron transport chain, particularly Complex I, as being highly targeted for desuccinylation by SIRT5...
April 30, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28456727/species-differences-in-the-binding-of-sodium-4-phenylbutyrate-to-serum-albumin
#15
Keishi Yamasaki, Taisuke Enokida, Kazuaki Taguchi, Shigeyuki Miyamura, Akito Kawai, Shuichi Miyamoto, Toru Maruyama, Hakaru Seo, Masaki Otagiri
Sodium 4-phenylbutyrate (PB) is clinically used as a drug for treating urea cycle disorders. Recent research has shown that PB also has other pharmacological activities, suggesting that it has the potential for use as a drug for treating other disorders. In the process of drug development, preclinical testing using experimental animals is necessary to verify the efficacy and safety of PB. While the binding of PB to human albumin has been studied, our knowledge of its binding to albumin from the other animal species is extremely limited...
April 26, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28444906/carbamoyl-phosphate-synthetase-1-deficiency-diagnosed-by-whole-exome-sequencing
#16
Guoqing Zhang, Yulin Chen, Huiqun Ju, Fei Bei, Jing Li, Jian Wang, Jianhua Sun, Jun Bu
BACKGROUND: Carbamoyl Phosphate Synthetase 1 deficiency (CPS1D) is a rare autosomal recessive inborn metabolic disease characterized mainly by hyperammonemia. The fatal nature of CPS1D and its similar symptoms with other urea cycle disorders (UCDs) make its diagnosis difficult, and the molecular diagnosis is hindered due to the large size of the causative gene CPS1. Therefore, the objective of the present study was to investigate the clinical applicability of exome sequencing in molecular diagnosis of CPS1D in Chinese population...
April 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28431974/unique-mitochondrial-localization-of-arginase-1-and-2-in-hepatocytes-of-air-breathing-walking-catfish-clarias-batrachus-and-their-differential-expression-patterns-under-hyper-ammonia-stress
#17
Bodhisattwa Banerjee, Debaprasad Koner, Priyanka Lal, Nirmalendu Saha
Arginase (ARG) catalyzes the final step of ornithine-urea cycle (OUC) leading to a conversion of L-arginine to L-ornithine and urea. Several isoforms of ARG have been reported in vertebrates, out of which the two predominant isoforms are the cytosolic ARG1 and the mitochondrial ARG2. The air-breathing walking catfish (Clarias batrachus) is frequently being challenged by different environmental insults such as hyper-ammonia, dehydration and osmotic stresses in their natural habitats throughout the year. The present study investigated the active presence of ARG1 and ARG2 isoforms in hepatocytes along with unique localization of both the isoforms inside the mitochondria, and also their specific expression patterns under hyper-ammonia stress (5mM NH4Cl) in isolated hepatocytes of walking catfish...
April 18, 2017: Gene
https://www.readbyqxmd.com/read/28425821/l-carnitine-supplementation-to-reverse-hyperammonemia-in-a-patient-undergoing-chronic-valproic-acid-treatment-a-case-report
#18
Cecilia Maldonado, Natalia Guevara, Alicia Silveira, Pietro Fagiolino, Marta Vázquez
Valproic acid is a broad-spectrum anticonvulsant that has also gained attention in the psychiatric setting. With respect to safety, valproic acid may induce a seemingly rare condition, hyperammonemia, which can induce a wide variety of symptoms ranging from irritability to coma. The proposed mechanism of hyperammonemia involves depletion of carnitine and overproduction of a toxic metabolite, 4-en-valproic acid, both of which impair the urea cycle and thus ammonia elimination. Carnitine is a commonly used antidote for acute intoxication of valproic acid, but is not a therapeutic option for management of chronic adults with adverse effects related to valproic acid...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28417441/relationships-between-essential-manganese-biology-and-manganese-toxicity-in-neurological-disease
#19
REVIEW
Anna C Pfalzer, Aaron B Bowman
PURPOSE OF REVIEW: Manganese (Mn) is critical for neurodevelopment but also has been implicated in the pathophysiology of several neurological diseases. We discuss how Mn requirements intersect with Mn biology and toxicity, and how these requirements may be altered in neurological disease. Furthermore, we discuss the emerging evidence that the level of Mn associated with optimal overall efficiency for Mn biology does not necessarily coincide with optimal cognitive outcomes. RECENT FINDINGS: Studies have linked Mn exposures with urea cycle metabolism and autophagy, with evidence that exposures typically neurotoxic may be able to correct deficiencies in these processes at least short term...
April 17, 2017: Current Environmental Health Reports
https://www.readbyqxmd.com/read/28394297/stepwise-splitting-growth-and-pseudocapacitive-properties-of-hierarchical-three-dimensional-co%C3%A2-o%C3%A2-nanobooks
#20
Huilong Chen, Shuang Lu, Feilong Gong, Huanzhen Liu, Feng Li
Three-dimensional hierarchical Co₃O₄ nanobooks have been synthesized successfully on a large scale by calcining orthorhombic Co(CO₃)0.5(OH)·0.11H₂O precursors with identical morphologies. Based on the influence of reaction time and urea concentration on the nanostructures of the precursors, a stepwise splitting growth mechanism can be proposed to understand the formation of the 3D nanobooks. The 3D Co₃O₄ nanobooks exhibit excellent pseudocapacitive performances with specific capacitances of 590, 539, 476, 453, and 421 F/g at current densities of 0...
April 10, 2017: Nanomaterials
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