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urea cycle

Soumen Bera, Sanjay Lamba, Mubasher Rashid, Anuj K Sharma, Alexander B Medvinsky, Claudia Acquisti, Amit Chakraborty, Bai-Lian Li
Impaired glutamate dehydrogenase (GDH) sensitivity to its inhibitors causes excessive insulin secretion by pancreatic beta-cells and defective ammonia metabolism in the liver. These symptoms are commonly associated with hyperinsulinism/hyperammonemia syndrome (HI/HA), which causes recurrent hypoglycaemia in early infancy. Hepatic localization of GDH amination and deamination activities linked with the urea cycle is known to be involved in ammonia metabolism and detoxification. Although deamination activities of hepatic GDH in the periportal zones of liver lobules and its connection to the urea cycle have been exhaustively investigated, physiological roles of GDH amination activity observed at pericentral zones have often been overlooked...
October 17, 2016: Integrative Biology: Quantitative Biosciences From Nano to Macro
Ruby Upadhyay, Thomas P Bleck, Katharina M Busl
Purpose. A 66-year-old man who presented with coma was found to have isolated severe hyperammonemia and diagnosed with a late-onset urea-cycle disorder. He was treated successfully and had full recovery. Methods. We report a novel case of noncirrhotic hyperammonemia and review the literature on this topic. Selected literature for review included English-language articles concerning hyperammonemia using the search terms "hyperammonemic encephalopathy", "non-cirrhotic encephalopathy", "hepatic encephalopathy", "urea-cycle disorders", "ornithine transcarbamylase (OTC) deficiency", and "fulminant hepatic failure"...
2016: Case Reports in Medicine
Cassiano Moro Piekarski, Antonio Carlos de Francisco, Leila Mendes da Luz, João Luiz Kovaleski, Diogo Aparecido Lopes Silva
Brazil is one of the largest producers of medium-density fibreboard (MDF) in the world, and also the MDF has the highest domestic consumption and production rate in the country. MDF applications are highlighted into residential and commercial furniture design and also a wide participation in the building sector. This study aimed to propose ways of improving the environmental cradle-to-gate life-cycle of one cubic meter MDF panel by means of a life-cycle assessment (LCA) study. Complying with requirements of ISO 14040 and 14,044 standards, different MDF manufacturing scenarios were modelled using Umberto® v...
October 10, 2016: Science of the Total Environment
Yuzheng Huang, Wei Li, Wuguang Lu, Chunrong Xiong, Yang Yang, Huaijiang Yan, Kun Connie Liu, Peng Cao
As one of the three major human pathogens that cause schistosomiasis, Schistosoma japonicum is the only one that is endemic in China. Despite great progress on schistosomiasis control over the past 50 years in China, S. japonicum transmission still occurs in certain endemic regions, which causes significant public health problems and enormous economic losses. During different life stages, parasites are able to survive dramatic osmolality changes between its vector, fresh water, and mammal host. However, the molecular mechanism of parasite osmoregulation remains unknown...
October 13, 2016: Scientific Reports
Zikria Saleem, Mobasher Ahmad, Furqan Khurshid Hashmi, Hamid Saeed, Muhammad Tahir Aziz
Doxorubicin and Cyclophosphamide (AC protocol) combination is usually considered as a first line therapy in newly diagnosed breast cancer patients. Thus, a retrospective observational study was conducted to monitor the effect of AC protocol on liver synthetic functions and production of plasma proteins in breast cancer patients, reporting to specialized cancer care hospital of Lahore, Pakistan. A total of 75 patients (n=75) on AC protocol with breast cancer were observed in this study. The patient data including age, gender, body surface area, dosage, disease status and laboratory biochemical values were recorded by reviewing historical treatment records...
September 2016: Pakistan Journal of Pharmaceutical Sciences
Emi Yamano, Masahiro Sugimoto, Akiyoshi Hirayama, Satoshi Kume, Masanori Yamato, Guanghua Jin, Seiki Tajima, Nobuhito Goda, Kazuhiro Iwai, Sanae Fukuda, Kouzi Yamaguti, Hirohiko Kuratsune, Tomoyoshi Soga, Yasuyoshi Watanabe, Yosky Kataoka
Chronic fatigue syndrome (CFS) is a persistent and unexplained pathological state characterized by exertional and severely debilitating fatigue, with/without infectious or neuropsychiatric symptoms, lasting at least 6 consecutive months. Its pathogenesis remains incompletely understood. Here, we performed comprehensive metabolomic analyses of 133 plasma samples obtained from CFS patients and healthy controls to establish an objective diagnosis of CFS. CFS patients exhibited significant differences in intermediate metabolite concentrations in the tricarboxylic acid (TCA) and urea cycles...
October 11, 2016: Scientific Reports
Jung Ju Choi, Hong Soon Kim, Kyung Cheon Lee, Youseok Shin, Youn Yi Jo
BACKGROUND: Citrullinemia type II is an autosomal recessive urea cycle disorder and a subtype of citrin deficiency. However, the management of recurrent hyperammonemia with neurologic symptoms in patients with citrullinemia type II is quite different from the management of other types of urea cycle disorders. In pats with citrullinemia type II, regional anesthesia might be a good choice for the early detection of hyperammonemic symptoms and addressing psychic stress. CASE PRESENTATION: A 48-year-old male with adult onset citrullinemia type II was scheduled for urethral scrotal fistula repair...
October 11, 2016: BMC Anesthesiology
Lukáš Novák, Zuzana Zubáčová, Anna Karnkowska, Martin Kolisko, Miluše Hroudová, Courtney W Stairs, Alastair G B Simpson, Patrick J Keeling, Andrew J Roger, Ivan Čepička, Vladimír Hampl
BACKGROUND: Multiple prokaryotic lineages use the arginine deiminase (ADI) pathway for anaerobic energy production by arginine degradation. The distribution of this pathway among eukaryotes has been thought to be very limited, with only two specialized groups living in low oxygen environments (Parabasalia and Diplomonadida) known to possess the complete set of all three enzymes. We have performed an extensive survey of available sequence data in order to map the distribution of these enzymes among eukaryotes and to reconstruct their phylogenies...
October 6, 2016: BMC Evolutionary Biology
Paul M K Gordon, Aneal Khan, Umair Sajid, Nicholas Chang, Varun Suresh, Leo Dimnik, Ryan E Lamont, Jillian S Parboosingh, Steven R Martin, Richard T Pon, Jene Weatherhead, Shelly Wegener, Debra Isaac, Steven C Greenway
Cell-free DNA (cfDNA) has significant potential in the diagnosis and monitoring of clinical conditions. However, accurately and easily distinguishing the relative proportion of DNA molecules in a mixture derived from two different sources (i.e., donor and recipient tissues after transplantation) is challenging. In human cellular transplantation, there is currently no useable method to detect in vivo engraftment, and blood-based non-invasive tests for allograft rejection in solid organ transplantation are either non-specific or absent...
2016: Frontiers in Cardiovascular Medicine
Eva P Pérez-Álvarez, Teresa Garde-Cerdán, Enrique García-Escudero, José María Martínez-Vidaurre
BACKGROUND: Nitrogen affects the grapevine growth and also the yeast metabolism, which influence directly on the fermentation kinetic and on the formation of different volatile compounds. Throughout grapevine cycle, soil nitrogen availability and grape nitrogen composition can vary as a consequence of different factors. Nitrogen foliar applications can contribute to enhance the grapevine nitrogen status and to minimize the problem of leaching that the traditional N-soil applications can provoke...
October 5, 2016: Journal of the Science of Food and Agriculture
Rahul Sadavarte, Carlos D M Filipe, Raja Ghosh
Heterologous overexpression of genes in Escherichia coli has made it possible to obtain high titers of recombinant proteins. However, this can result in the formation of aggregated protein particles known as 'inclusion bodies'. Protein sequestered as inclusion body is inactive and needs to be converted back to its functional form by refolding using appropriate techniques. In the current study inclusion bodies of the enzyme aminoglycoside nucleotidyl transferase (or ANT(2")-Ia) were first solubilized in urea and subsequently subjected to thermal-cycling under controlled conditions as part of the refolding strategy...
September 30, 2016: Biotechnology Progress
Rui-Ping Sun, Qian-Yun Xi, Jia-Jie Sun, Xiao Cheng, Yan-Ling Zhu, Ding-Ze Ye, Ting Chen, Li-Min Wei, Rui-Song Ye, Qing-Yan Jiang, Yong-Liang Zhang
Ammonia detoxification, which takes place via the hepatic urea cycle, is essential for nitrogen homeostasis and physiological well-being. It has been reported that a reduction in dietary protein reduces urea nitrogen. MicroRNAs (miRNAs) are major regulatory non-coding RNAs that have significant effects on several metabolic pathways; however, little is known on whether miRNAs regulate hepatic urea synthesis. The objective of this study was to assess the miRNA expression profile in a low protein diet and identify miRNAs involved in the regulation of the hepatic urea cycle using a porcine model...
September 30, 2016: Scientific Reports
Illa Tea, Estelle Martineau, Ingrid Antheaume, Julie Lalande, Caroline Mauve, Francoise Gilard, Sophie Barillé-Nion, Anneke C Blackburn, Guillaume Tcherkez
Breast cancer is the most common cancer in women worldwide. Despite the information provided by anatomopathological assessment and molecular markers (such as receptor expression ER, PR, HER2), breast cancer therapies and prognostics depend on the metabolic properties of tumor cells. However, metabolomics have not provided a robust and congruent biomarker yet, likely because individual metabolite contents are insufficient to encapsulate all of the alterations in metabolic fluxes. Here, we took advantage of natural (13)C and (15)N isotope abundance to show there are isotopic differences between healthy and cancer biopsy tissues or between healthy and malignant cultured cell lines...
September 28, 2016: Scientific Reports
F-C Tang, C-C Chan
BACKGROUND/OBJECTIVES: Branched-chain amino acids (BCAAs) and purine nucleotide cycle (PNC) are both associated with energy metabolism. The purpose of this study was to explore the influences of BCAA supplementation on the PNC activity of male athletes in response to a bout of endurance running exercise. SUBJECTS/METHODS: Twelve male athletes (20.3±1.4 years) participated in the study. Each of the athletes received 12 g of a BCAA supplement (leucine 54%, isoleucine 19% and valine 27%) per day during the study...
September 28, 2016: European Journal of Clinical Nutrition
Eliza Mnich, Magdalena Kowalewicz-Kulbat, Paulina Sicińska, Krzysztof Hinc, Michał Obuchowski, Adrian Gajewski, Anthony P Moran, Magdalena Chmiela
AIM: To determine the impact of selected well defined Helicobacter pylori (H. pylori) antigens on gastric barrier cell turnover. METHODS: In this study, using two cellular models of gastric epithelial cells and fibroblasts, we have focused on exploring the effects of well defined H. pylori soluble components such as glycine acid extract antigenic complex (GE), subunit A of urease (UreA), cytotoxin associated gene A protein (CagA) and lipopolysaccharide (LPS) on cell turnover by comparing the wound healing capacity of the cells in terms of their proliferative and metabolic activity as well as cell cycle distribution...
September 7, 2016: World Journal of Gastroenterology: WJG
Marie-Caroline Husson, Manuel Schiff, Alain Fouilhoux, Aline Cano, Dries Dobbelaere, Anais Brassier, Karine Mention, Jean-Baptiste Arnoux, François Feillet, Brigitte Chabrol, Nathalie Guffon, Caroline Elie, Pascale de Lonlay
BACKGROUND: The efficacy and safety of intra-venous (i.v.) sodium benzoate for treating acute episodes of hyperammonemia in urea cycle enzyme disorders (UCD) is well known. However, published data do not provide a clear picture of the benefits and risks of this drug. We report a retrospective multicentre study on the use of i.v. sodium benzoate in patients treated for UCD between 2000 and 2010 in the 6 French reference centres for metabolic diseases. RESULTS: Sixty-one patients with UCDs - 22 ornithine transcarbamylase (20 confirmed, 2 suspected), 18 arginino-succinate synthetase, 15 carbamoyl phosphate synthetase, 3 arginosuccinate lyase, 1 arginase deficiency, 1 N-acetylglutamate synthetase, 1 HHH syndrome - required i...
2016: Orphanet Journal of Rare Diseases
Daniela Proverbio, Eva Spada, Giada Bagnagatti de Giorgi, And Roberta Perego
AIM: The aim of this study was to evaluate changes in proteinuria in dogs naturally infected with visceral leishmaniasis, following treatment with miltefosine (MLF) and allopurinol. MATERIALS AND METHODS: Medical records of 40 dogs with leishmaniasis, treated with 2 mg/kg MLF every 24 h PO and 10 mg/kg allopurinol every 12 h for 28 days were reviewed. 20 dogs were included in the study, and clinical staging was performed following guidelines of the Canine leishmaniasis (CanL) Working Group, and dogs were categorized for proteinuria according to the International Renal Interest Society (IRIS) staging system...
August 2016: Veterinary World
D M Rotroff, D G Corum, A Motsinger-Reif, O Fiehn, N Bottrel, W C Drevets, J Singh, G Salvadore, R Kaddurah-Daouk
Ketamine, at sub-anesthetic doses, is reported to rapidly decrease depression symptoms in patients with treatment-resistant major depressive disorder (MDD). Many patients do not respond to currently available antidepressants, (for example, serotonin reuptake inhibitors), making ketamine and its enantiomer, esketamine, potentially attractive options for treatment-resistant MDD. Although mechanisms by which ketamine/esketamine may produce antidepressant effects have been hypothesized on the basis of preclinical data, the neurobiological correlates of the rapid therapeutic response observed in patients receiving treatment have not been established...
2016: Translational Psychiatry
Yehouda Marcus, Noam Tal, Mordechai Ronen, Raanan Carmieli, Michael Gurevitz
Ornidazole of the 5-nitroimidazole drug family is used to treat protozoan and anaerobic bacterial infections via a mechanism that involves pre-activation by reduction of the nitro group, and production of toxic derivatives and radicals. Metronidazole, another drug family member, has been suggested to affect photosynthesis by draining electrons from the electron carrier, ferredoxin, thus inhibiting NADP(+) reduction and stimulating radical and peroxide production. Here we show, however, that ornidazole inhibits photosynthesis via a different mechanism...
September 19, 2016: Biochemical Journal
Theodorus B M Hakvoort, Youji He, Wim Kulik, Jacqueline L M Vermeulen, Suzanne Duijst, Jan M Ruijter, Jurgen H Runge, Nicolaas E P Deutz, S Eleonore Koehler, Wouter H Lamers
: Glutamine synthetase (GS) catalyzes condensation of ammonia with glutamate to glutamine. Glutamine serves, with alanine, as major nontoxic interorgan ammonia carrier. Elimination of hepatic GS expression in mice causes only mild hyperammonemia and hypoglutaminemia, but a pronounced decrease in the whole-body muscle-to-fat ratio with increased myostatin expression in muscle. Using GS-KO/Liver and control mice, and stepwise increments of enterally infused ammonia, we show that ∼35% of this ammonia is detoxified by hepatic GS and ∼35% by urea-cycle enzymes, while ∼30% is not cleared by the liver, independent of portal ammonia concentrations ≤2 mmol/L...
September 19, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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