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https://www.readbyqxmd.com/read/28444906/carbamoyl-phosphate-synthetase-1-deficiency-diagnosed-by-whole-exome-sequencing
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/28393236/metabolomic-analysis-of-the-saliva-of-japanese-patients-with-oral-squamous-cell-carcinoma
#6
Mitsuyoshi Ohshima, Keisuke Sugahara, Kiyohiro Kasahara, Akira Katakura
The aim of the present study was to characterize the metabolic systems in Japanese patients with oral squamous cell carcinoma (OSCC) using capillary electrophoresis-mass spectrometry (CE-MS) metabolome analysis of saliva samples. A previous study showed variations among ethnicities and tumor sites in the saliva metabolome of patients with OSCC using CE-MS. In the present study, saliva was obtained from 22 Japanese patients with OSCC and from 21 healthy controls who visited the Department of Dentistry, Oral and Maxillofacial Surgery, Tokyo Dental Collage Ichikawa General Hospital, Tokyo, Japan, and all samples were subject to comprehensive quantitative metabolome analysis using CE-MS...
April 6, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28382234/comparative-proteomics-of-rhizopus-delemar-atcc-20344-unravels-the-role-of-amino-acid-catabolism-in-fumarate-accumulation
#7
Dorett I Odoni, Juan A Tamayo-Ramos, Jasper Sloothaak, Ruben G A van Heck, Vitor A P Martins Dos Santos, Leo H de Graaff, Maria Suarez-Diez, Peter J Schaap
The filamentous fungus Rhizopus delemar naturally accumulates relatively high amounts of fumarate. Although the culture conditions that increase fumarate yields are well established, the network underlying the accumulation of fumarate is not yet fully understood. We set out to increase the knowledge about fumarate accumulation in R. delemar. To this end, we combined a transcriptomics and proteomics approach to identify key metabolic pathways involved in fumarate production in R. delemar, and propose that a substantial part of the fumarate accumulated in R...
2017: PeerJ
https://www.readbyqxmd.com/read/28376202/role-of-cps1-in-cell-growth-metabolism-and-prognosis-in-lkb1-inactivated-lung-adenocarcinoma
#8
Müge Çeliktas, Ichidai Tanaka, Satyendra Chandra Tripathi, Johannes F Fahrmann, Clemente Aguilar-Bonavides, Pamela Villalobos, Oliver Delgado, Dilsher Dhillon, Jennifer B Dennison, Edwin J Ostrin, Hong Wang, Carmen Behrens, Kim-Anh Do, Adi F Gazdar, Samir M Hanash, Ayumu Taguchi
Background: Liver kinase B1 ( LKB1 ) is a tumor suppressor in lung adenocarcinoma (LADC). We investigated the proteomic profiles of 45 LADC cell lines with and without LKB1 inactivation. Carbamoyl phosphate synthetase 1 (CPS1), the first rate-limiting mitochondrial enzyme in the urea cycle, was distinctively overexpressed in LKB1-inactivated LADC cell lines. We therefore assessed the role of CPS1 and its clinical relevance in LKB1-inactivated LADC. Methods: Mass spectrometric profiling of proteome and metabolome and function of CPS1 were analyzed in LADC cell lines...
March 1, 2017: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/28367300/renal-and-hepatic-functions-after-a-week-of-controlled-ovarian-hyperstimulation-during-in-vitro-fertilization-cycles
#9
Ilaria Romito, Ferdinando Antonio Gulino, Antonio Simone Laganà, Salvatore Giovanni Vitale, Attilio Tuscano, Gianluca Leanza, Giulia Musmeci, Vito Leanza, Agnese Maria Chiara Rapisarda, Marco Antonio Palumbo
BACKGROUND: One the main aspects of in vitro fertilization (IVF) cycle is to avoid any possible systemic damage on women undergoing a controlled ovarian hyperstimulation (COH). The aim of this work is to evaluate renal and hepatic function blood tests in patients undergoing controlled ovarian hyperstimulation during IVF cycles. MATERIALS AND METHODS: We performed a prospective cohort analysis. All patients re- ceived a long stimulation protocol with gonadotropin-releasing hormone (GnRH) analogues by daily administration, since the twenty-first day of the previous ovarian cycle followed by COH with recombinant follicle-stimulating hormone (FSH)...
April 2017: International Journal of Fertility & Sterility
https://www.readbyqxmd.com/read/28351018/profiles-of-amino-acids-and-biogenic-amines-in-the-plasma-of-cri-du-chat-patients
#10
Danielle Zildeana Sousa Furtado, Fernando Brunale Vilela de Moura Leite, Cleber Nunes Barreto, Bernadete Faria, Leticia Dias Lima Jedlicka, Elisângela de Jesus Silva, Heron Dominguez Torres da Silva, Etelvino Jose Henriques Bechara, Nilson Antonio Assunção
Cri-du-chat syndrome (CDCS) is a rare innate disease attributed to chromosome 5p deletion characterized by a cat-like cry, craniofacial malformation, and altered behavior of affected children. Metabolomic analysis and a chemometric approach allow description of the metabolic profile of CDCS as compared to normal subjects. In the present work, UHPLC/MS was employed to analyze blood samples withdrawn from CDCS carriers (n=18) and normal parental subjects (n=18), all aged 0-34 years, aiming to set up a representative CDCS profile constructed from 33 targeted amino acids and biogenic amines...
June 5, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28338677/integrated-metabolism-in-sponge-microbe-symbiosis-revealed-by-genome-centered-metatranscriptomics
#11
Lucas Moitinho-Silva, Cristina Díez-Vives, Giampiero Batani, Ana Is Esteves, Martin T Jahn, Torsten Thomas
Despite an increased understanding of functions in sponge microbiomes, the interactions among the symbionts and between symbionts and host are not well characterized. Here we reconstructed the metabolic interactions within the sponge Cymbastela concentrica microbiome in the context of functional features of symbiotic diatoms and the host. Three genome bins (CcPhy, CcNi and CcThau) were recovered from metagenomic data of C. concentrica, belonging to the proteobacterial family Phyllobacteriaceae, the Nitrospira genus and the thaumarchaeal order Nitrosopumilales...
March 24, 2017: ISME Journal
https://www.readbyqxmd.com/read/28330145/enhanced-xylitol-production-using-immobilized-candida-tropicalis-with-non-detoxified-corn-cob-hemicellulosic-hydrolysate
#12
Tatyaso Yewale, Shruti Panchwagh, Srinivasan Rajagopalan, Pradip B Dhamole, Rishi Jain
This study reports an industrially applicable non-sterile xylitol fermentation process to produce xylitol from a low-cost feedstock like corn cob hydrolysate as pentose source without any detoxification. Different immobilization matrices/mediums (alginate, polyvinyl alcohol, agarose gel, polyacrylamide, gelatin, and κ-carrageenan) were studied to immobilize Candida tropicalis NCIM 3123 cells for xylitol production. Amongst this calcium alginate, immobilized cells produced maximum amount of xylitol with titer of 11...
June 2016: 3 Biotech
https://www.readbyqxmd.com/read/28325852/extended-multiplexing-of-tmt-labeling-reveals-age-and-high-fat-diet-specific-proteome-changes-in-mouse-epididymal-adipose-tissue
#13
Deanna L Plubell, Phillip A Wilmarth, Yuqi Zhao, Alexandra M Fenton, Jessica Minnier, Ashok P Reddy, John Klimek, Xia Yang, Larry L David, Nathalie Pamir
The lack of high-throughput methods to analyze the adipose tissue protein composition limits our understanding of the protein networks responsible for age and diet related metabolic response. We have developed an approach using multiple-dimension liquid chromatography tandem mass spectrometry and extended multiplexing (24 biological samples) with TMT labeling to analyze proteomes of epididymal adipose tissues isolated from mice fed either low or high fat diet for a short or a long-term, and from mice that aged on low vs...
March 21, 2017: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/28323949/plasma-arginine-asymmetric-dimethylarginine-ratio-and-incidence-of-cardiovascular-events-a-case-cohort-study
#14
Edward Yu, Miguel Ruiz-Canela, Frank B Hu, Clary B Clish, Dolores Corella, Jordi Salas-Salvadó, Adela Hruby, Montserrat Fitó, Liming Liang, Estefania Toledo, Emilio Ros, Ramón Estruch, Enrique Gómez-Gracia, Jose Lapetra, Fernando Arós, Dora Romaguera, Lluís Serra-Majem, Marta Guasch-Ferré, Dong D Wang, Miguel A Martínez-González
CONTEXT: Arginine, its methylated metabolites and other metabolites related to the urea cycle have been independently associated with cardiovascular risk, but the potential causal meaning of these associations (positive for some metabolites and negative for others) remains elusive due to a lack of studies measuring metabolite changes over time. OBJECTIVE: To examine the association between baseline and 1-year concentrations of urea cycle metabolites and cardiovascular disease in a case-cohort setting...
March 2, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28319841/mesoporous-magnesium-oxide-nanoparticles-derived-via-complexation-combustion-for-enhanced-performance-in-carbon-dioxide-capture
#15
Vishwanath Hiremath, Raghavendra Shavi, Jeong Gil Seo
Magnesium oxide (MgO) is a promising candidate for carbon dioxide (CO2) capture at high temperature applicable to pre-combustion capture in an integrated gasification combined cycle (IGCC) scheme. In this work, mesoporous MgO nanoparticles were synthesized via simple complexation-combustion method by using glycine (G) and urea (U) as fuels (F). The obtained sorbents were thoroughly characterized in terms of the crystalline structure, morphology, nature of the fuel, F/O ratio, and their consequent effects on CO2 sorption...
March 10, 2017: Journal of Colloid and Interface Science
https://www.readbyqxmd.com/read/28319373/morphology-controllable-synthesis-of-zn-co-mixed-sulfide-nanostructures-on-carbon-fiber-paper-toward-efficient-rechargeable-zinc-air-batteries-and-water-electrolysis
#16
Xiaoyu Wu, Xiaopeng Han, Xiaoya Ma, Wei Zhang, Yida Deng, Cheng Zhong, Wenbin Hu
It remains an ongoing challenge to develop cheap, highly active, and stable electrocatalysts to promote the sluggish electrocatalytic oxygen evolution, oxygen reduction, and hydrogen evolution reactions for rechargeable metal-air batteries and water-splitting systems. In this work, we report the morphology-controllable synthesis of zinc cobalt mixed sulfide (Zn-Co-S) nanoarchitectures, including nanosheets, nanoplates, and nanoneedles, grown on conductive carbon fiber paper (CFP) and the micronanostructure dependent electrochemical efficacy for catalyzing hydrogen and oxygen in zinc-air batteries and water electrolysis...
March 28, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28302489/modelling-urea-cycle-disorder-citrullinemia-type-1-with-disease-specific-ipscs
#17
Elena Yukie Yoshitoshi-Uebayashi, Taro Toyoda, Katsutaro Yasuda, Maki Kotaka, Keiko Nomoto, Keisuke Okita, Kentaro Yasuchika, Shinya Okamoto, Noriyuki Takubo, Toshiya Nishikubo, Tomoyoshi Soga, Shinji Uemoto, Kenji Osafune
Citrullinemia type 1 (CTLN1) is a urea cycle disorder (UCD) caused by mutations of the ASS1 gene, which is responsible for production of the enzyme argininosuccinate synthetase (ASS), and classically presented as life-threatening hyperammonemia in newborns. Therapeutic options are limited, and neurological sequelae may persist. To understand the pathophysiology and find novel treatments, induced pluripotent stem cells (iPSCs) were generated from a CTLN1 patient and differentiated into hepatocyte-like cells (HLCs)...
March 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28296339/controlling-the-hydrolysis-and-loss-of-nitrogen-fertilizer-urea-using-a-nanocomposite
#18
Naiqin Zhong, Linglin Zhou, Pan Zhao, Yu Chi, Dongfang Wang, Pan Wang, Ning Liu, Dongqing Cai, Zhengyan Wu
Urea tends to be hydrolyzed by urease and then migrate to the environment, resulting in low utilization efficiency and severe environmental contamination. To solve this problem, a fertilizer synergist (FS) was developed using sodium humate transported by a nanonetwork through hydrogen bonds. FS could effectively inhibit the hydrolysis, reduce the loss amount, and enhance the UE of nitrogen. Furthermore, FS exerted significant positive effects on the expression of several nitrogen uptake-related genes, ion flux in maize roots, the growth of crops and the organic matter in soil...
March 10, 2017: ChemSusChem
https://www.readbyqxmd.com/read/28288441/carbon-footprint-as-an-environmental-sustainability-indicator-for-the-particleboard-produced-in-pakistan
#19
Majid Hussain, Riffat Naseem Malik, Adam Taylor
This study quantified the carbon footprint of particleboard production in Pakistan using a cradle-to-gate life cycle assessment approach. The system boundary comprised raw materials acquisition, transport, particleboard manufacture and finished product distribution. Primary data were collected through surveys and meetings with particleboard manufacturers. Secondary data were taken from the literature. Greenhouse gas emissions from off-site industrial operations of the particleboard industry represented 52% of the total emissions from the production of 1...
May 2017: Environmental Research
https://www.readbyqxmd.com/read/28284393/epilepsy-in-inborn-errors-of-metabolism-with-therapeutic-options
#20
Jaume Campistol
Inborn errors of metabolism (IEM) are rare conditions that represent more than 1000 diseases, with a global prevalence of approximately 1:2000 individuals. Approximately, 40%-60% of IEM may present with epilepsy as one of the main neurologic signs. Epilepsy in IEM may appear at any age (fetal, newborn, infant, adolescent, or even adult). Different pathophysiological mechanisms may be responsible for the clinical phenotype, such as disturbances in energy metabolism (mitochondrial and fatty oxidation disorders, GLUT-1, and cerebral creatine deficiency), accumulation of complex molecules (lysosomal storage disorders), toxic mechanisms (organic acidurias and urea cycle disorders), or impairment of neurotransmission...
November 2016: Seminars in Pediatric Neurology
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