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https://www.readbyqxmd.com/read/28213125/reduced-bioavailable-manganese-causes-striatal-urea-cycle-pathology-in-huntington-s-disease-mouse-model
#1
Terry Jo V Bichell, Michal Wegrzynowicz, K Grace Tipps, Emma M Bradley, Michael A Uhouse, Miles Bryan, Kyle Horning, Nicole Fisher, Karrie Dudek, Timothy Halbesma, Preethi Umashanker, Andrew D Stubbs, Hunter K Holt, Gunnar F Kwakye, Andrew M Tidball, Roger J Colbran, Michael Aschner, M Diana Neely, Alba Di Pardo, Vittorio Maglione, Alexander Osmand, Aaron B Bowman
Huntington's disease (HD) is caused by a mutation in the huntingtin gene (HTT), resulting in profound striatal neurodegeneration through an unknown mechanism. Perturbations in the urea cycle have been reported in HD models and in HD patient blood and brain. In neurons, arginase is a central urea cycle enzyme, and the metal manganese (Mn) is an essential cofactor. Deficient biological responses to Mn, and reduced Mn accumulation have been observed in HD striatal mouse and cell models. Here we report in vivo and ex vivo evidence of a urea cycle metabolic phenotype in a prodromal HD mouse model...
February 14, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28212266/individual-patterns-in-blood-borne-indicators-of-fatigue-trait-or-chance
#2
Ross Julian, Tim Meyer, Hugh H K Fullagar, Sabrina Skorski, Mark Pfeiffer, Michael Kellmann, Alexander Ferrauti, Anne Hecksteden
Julian, R, Meyer, T, Fullagar, HHK, Skorski, S, Pfeiffer, M, Kellmann, M, Ferrauti, A, and Hecksteden, A. Individual patterns in blood-borne indicators of fatigue-trait or chance. J Strength Cond Res 31(3): 608-619, 2017-Blood-borne markers of fatigue such as creatine kinase (CK) and urea (U) are widely used to fine-tune training recommendations. However, predictive accuracy is low. A possible explanation for this dissatisfactory characteristic is the propensity of athletes to react to different patterns of fatigue indicators (e...
March 2017: Journal of Strength and Conditioning Research
https://www.readbyqxmd.com/read/28205048/mild-orotic-aciduria-in-umps-heterozygotes-a-metabolic-finding-without-clinical-consequences
#3
Saskia B Wortmann, Margaret A Chen, Roberto Colombo, Alessandro Pontoglio, Bader Alhaddad, Lorenzo D Botto, Tatiana Yuzyuk, Curtis R Coughlin, Maria Descartes, Stephanie Grűnewald, Bruno Maranda, Philippa B Mills, James Pitt, Catherine Potente, Richard Rodenburg, Leo A J Kluijtmans, Srirangan Sampath, Emil F Pai, Ron A Wevers, George E Tiller
BACKGROUND: Elevated urinary excretion of orotic acid is associated with treatable disorders of the urea cycle and pyrimidine metabolism. Establishing the correct and timely diagnosis in a patient with orotic aciduria is key to effective treatment. Uridine monophosphate synthase is involved in de novo pyrimidine synthesis. Uridine monophosphate synthase deficiency (or hereditary orotic aciduria), due to biallelic mutations in UMPS, is a rare condition presenting with megaloblastic anemia in the first months of life...
February 15, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28187035/hepatocyte-antigen-expression-in-barrett-esophagus-and-associated-neoplasia
#4
Sejal S Shah, Vishal S Chandan
Hepatocyte antigen or hepatocyte paraffin 1 (Hep Par 1) is widely used as a diagnostic immunomarker for hepatocellular carcinoma. It has also been identified as a rate-limiting enzyme of the urea cycle, carbamoyl phosphate synthetase 1. Hep Par 1 has been detected in non-neoplastic small intestinal epithelium, but its expression in Barrett esophagus and its related neoplasia has not been well investigated. We immunohistochemically evaluated expression of Hep Par 1 on 75 cases of Barrett esophagus (25 cases without dysplasia, 16 cases with low-grade dysplasia, 25 cases with high-grade dysplasia, and 9 cases with intramucosal adenocarcinoma) on endoscopic biopsies and endoscopic mucosal resections...
February 9, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28182865/restoration-of-immune-and-renal-function-in-aged-females-by-re-establishment-of-active-ovarian-function
#5
Rhett L Peterson, Kate C Parkinson, Jeffrey B Mason
Proper immune functioning is necessary to maximize reproductive success. In addition, age-associated uremia in women is often associated with hypothalamic--pituitary-gonadal dysfunction. In the present experiments, we tested immune and renal function to determine if exposure of postreproductive mice to young, reproductively cycling ovaries would influence non-reproductive physiological functions. Control female CBA/J mice were evaluated at 6, 13 and 16 months of age. Additional mice received new (60-day-old) ovaries at 12 months of age and were evaluated at 16 months of age...
February 10, 2017: Reproduction, Fertility, and Development
https://www.readbyqxmd.com/read/28179395/molecular-physiology-of-freeze-tolerance-in-vertebrates
#6
REVIEW
Kenneth B Storey, Janet M Storey
Freeze tolerance is an amazing winter survival strategy used by various amphibians and reptiles living in seasonally cold environments. These animals may spend weeks or months with up to ∼65% of their total body water frozen as extracellular ice and no physiological vital signs, and yet after thawing they return to normal life within a few hours. Two main principles of animal freeze tolerance have received much attention: the production of high concentrations of organic osmolytes (glucose, glycerol, urea among amphibians) that protect the intracellular environment, and the control of ice within the body (the first putative ice-binding protein in a frog was recently identified), but many other strategies of biochemical adaptation also contribute to freezing survival...
April 2017: Physiological Reviews
https://www.readbyqxmd.com/read/28168106/untargeted-metabolomics-approach-for-unraveling-robust-biomarkers-of-nutritional-status-in-fasted-gilthead-sea-bream-sparus-aurata
#7
Ruben Gil-Solsona, Jaime Nácher-Mestre, Leticia Lacalle-Bergeron, Juan Vicente Sancho, Josep Alvar Calduch-Giner, Félix Hernández, Jaume Pérez-Sánchez
A metabolomic study has been performed to identify sensitive and robust biomarkers of malnutrition in farmed fish, using gilthead sea bream (Sparus aurata) as a model. The metabolomic fingerprinting of serum from fasted fish was assessed by means of ultra-high performance liquid chromatography coupled to quadrupole time-of-flight mass spectrometry. More than 15,000 different m/z ions were detected and Partial Least Squares-Discriminant analysis allowed a clear differentiation between the two experimental groups (fed and 10-day fasted fish) with more than 90% of total variance explained by the two first components...
2017: PeerJ
https://www.readbyqxmd.com/read/28167158/most-individuals-with-advanced-cirrhosis-have-sleep-disturbances-which-are-associated-with-poor-quality-of-life
#8
Marwan Ghabril, Mollie Jackson, Raghavender Gotur, Regina Weber, Eric Orman, Raj Vuppalanchi, Naga Chalasani
BACKGROUND & AIMS: Sleep disturbances are common in patients with cirrhosis, but their determinants and effects on health-related quality of life are not well understood. We investigated the prevalence of disturbed sleep in these patients, factors associated with sleep disruption, and effects on quality of life. METHODS: We performed a prospective, cross-sectional study of 193 stable ambulatory patients with cirrhosis (154 with decompensated cirrhosis). Participants completed the Pittsburgh sleep questionnaire index (to assess sleep quality), the chronic liver disease questionnaire (CLDQ), muscle cramp questionnaires, and underwent neurocognitive testing...
February 3, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28164183/a-rechargeable-al-ion-battery-al-molten-alcl3-urea-graphite
#9
Handong Jiao, Chen Wang, Jiguo Tu, Donghua Tian, Shuqiang Jiao
A new Al-ion battery based on an affordable and nontoxic liquid electrolyte made from molten AlCl3/urea was assembled. As the cathode material, natural graphite shows two well-defined discharge voltage plateaus at about 1.9 and 1.5 V with a high specific capacity of 93 mA h g(-1) and excellent coulombic efficiency (>99%). The attractive capacity (about 78 mA h g(-1)) is retained even at a high current density of 1000 mA g(-1). Moreover, no faster fading in capacity is observed after 500 cycles. This electrolyte could provide a new system for Al ion batteries, which can be used for large scale energy storage, owing to its cost advantages, high-rate capability and durability...
February 6, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28157284/mechanisms-of-increased-particle-and-voc-emissions-during-dpf-active-regeneration-and-practical-emissions-considering-regeneration
#10
Hiroyuki Yamada, Satoshi Inomata, Hiroshi Tanimoto
Mechanisms involved in increased particle and volatile organic compound (VOC) emissions during active and parked active regenerations of a diesel particulate filter (DPF) were investigated using heavy-duty trucks equipped with both a urea selective catalytic reduction system and a DPF (SCR + DPF) and a DPF-only. Particle emissions increased in the later part of the regeneration period but the mechanisms were different above and below 23 nm. Particles above 23 nm were emitted due to the lower filtering efficiency of the DPF because of the decreasing amount of soot trapped during regeneration...
February 3, 2017: Environmental Science & Technology
https://www.readbyqxmd.com/read/28151661/an-efficient-reversible-hydrogen-carrier-system-based-on-amine-reforming-of-methanol
#11
Jotheeswari Kothandaraman, Sayan Kar, Raktim Sen, Alain Goeppert, George A Olah, G K Surya Prakash
A novel hydrogen storage system based on the hydrogen release from catalytic dehydrogenative coupling of methanol and 1,2-diamine is demonstrated. The products of this reaction, N-formamide and N,N'-diformamide, are hydrogenated back to the free amine and methanol by simple hydrogen pressure swing. Thus, an efficient one-pot hydrogen carrier system has been developed. The H2 generating step can be termed as "amine reforming of methanol" in analogy to the traditional steam reforming of methanol. It acts as a clean source of hydrogen without concurrent production of CO2 (unlike steam reforming) or CO (by complete methanol dehydrogenation)...
February 2, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28149015/role-of-bioflavonoid-quercetin-on-expression-of-urea-cycle-enzymes-astrocytic-and-inflammatory-markers-in-hyperammonemic-rats
#12
Sivamani Kanimozhi, Perumal Subramanian, Sakkaravarthy Shanmugapriya, Subramanian Sathishkumar
This study evaluates the role of quercetin on the expression of urea cycle enzymes, astrocytic, neuronal and inflammatory markers in hyperammonemic rats. Hyperammonemia (provoked by intraperitonial injections of (ammonium chloride-100 mg/kg b.w for 56 days), showed diminished expression of urea cycle enzymes [carbamyl phosphate synthetase-1 (CPS-1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS) and arginase (ARG)] in liver and decreased expression of neuronal and astrocytic markers-glutamine synthase (GS) and phosphate activated glutaminase (PAG) in brain and increased expression of brain inflammatory markers such as interleukin 6 (IL6), inducible nitric oxide synthase (iNOS) and nuclear transcription factor kappa B (NF-κB) (by western blot analysis) and exhibited downregulated expression of soluble guanylate cyclase (sGC), glial fibrillary acidic protein (GFAP) in brain and ASS in liver investigated (by RT-PCR)...
March 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28142125/potential-role-of-compost-and-green-manure-amendment-to-mitigate-soil-ghgs-emissions-in-mediterranean-drip-irrigated-maize-production-systems
#13
Annachiara Forte, Massimo Fagnano, Angelo Fierro
Organic fertilization can preserve soil organic matter (SOM) and is foreseen as an effective strategy to reduce green house gases (GHGs) emissions in agriculture. However, its effectiveness needs to be clarified under specific climate, crop management and soil characteristics. A field experiment was carried out in a Mediterranean drip irrigated maize system to assess the pattern of soil CO2 and N2O fluxes in response to the replacement of a typical bare fallow-maize cycle under urea fertilization (130 kg N ha(-1) y(-1)) (CONV) with: (i) bare fallow-maize cycles under two doses of compost (COM1 and COM2, 130 and 260 kg N ha(-1) y(-1), respectively) and (ii) a vetch-maize cycle, with vetch incorporation as green manure (130 kg N ha(-1) y(-1)) (GMAN)...
January 28, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/28130067/farnesoid-x-receptor-activation-promotes-hepatic-amino-acid-catabolism-and-ammonium-clearance-in-mice
#14
Vittoria Massafra, Alexandra Milona, Harmjan R Vos, Rúben J J Ramos, Johan Gerrits, Ellen C L Willemsen, José M Ramos Pittol, Noortje Ijssennagger, Martin Houweling, Hubertus C M T Prinsen, Nanda M Verhoeven-Duif, Boudewijn M Burgering, Saskia W C van Mil
BACKGROUND & AIMS: The nuclear receptor subfamily 1 group H member 4 (NR1H4 or farnesoid X receptor, FXR) regulates bile acid synthesis, transport, and catabolism. FXR also regulates post-prandial lipid and glucose metabolism. We performed quantitative proteomic analyses of liver tissues from mice to evaluate these functions and investigate whether FXR regulates amino acid metabolism. METHODS: To study the role of FXR in mouse liver, we used mice with a disruption of Nr1h4 (FXR-knockout mice) and compared them to floxed control mice...
January 24, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28129265/parenteral-and-enteral-nutrition-in-surgical-critical-care-plasma-metabolomics-demonstrates-divergent-effects-on-nitrogen-fatty-acid-ribonucleotide-and-oxidative-metabolism
#15
Brodie A Parent, Max Seaton, Danijel Djukovic, Haiwei Gu, Brittany Wheelock, Daniel Raftery, Grant E O'Keefe
BACKGROUND: Artificial nutrition support is central to the care of critically ill patients and is primarily provided enterally (EN). There are circumstances when parenteral nutrition (PN) is considered necessary. We are uncertain how each of these approaches confer clinical benefits beyond simply providing calories. We sought to better understand how each of these techniques influence metabolism in critically-ill patients using a broad-based metabolomics approach. Metabolic responses to EN and PN may differ in ways that could help us understand how to optimize use of these therapies...
January 26, 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28127796/construction-of-bioengineered-hepatic-tissue-derived-from-human-umbilical-cord-mesenchymal-stem-cells-via-aggregation-culture-in-porcine-decellularized-liver-scaffolds
#16
Yi Li, Qiong Wu, Yujia Wang, Li Li, Fei Chen, Yujun Shi, Ji Bao, Hong Bu
BACKGROUND: An individualized, tissue-engineered liver suitable for transplanting into a patient with liver disease would be of great benefit to the patient and the healthcare system. The tissue-engineered liver would possess the functions of the original healthy organ. Two fields of study, (i) using decellularized tissue as cell scaffolding, and (ii) stem cell differentiation into functional cells, are coming together to make this concept feasible. The decellularized liver scaffolds (DLS) can interact with cells to promote cell differentiation and signal transduction and three-dimensional (3D) stem cell aggregations can maintain the phenotypes and improve functions of stem cells after differentiation by undergoing cell-cell contact...
January 26, 2017: Xenotransplantation
https://www.readbyqxmd.com/read/28122810/testosterone-prevents-protein-loss-via-the-hepatic-urea-cycle-in-human
#17
Teresa Lam, Anne Poljak, Mark McLean, Neha Bahl, Ken K Y Ho, Vita Birzniece
CONTEXT: The urea cycle is a rate limiting step for amino acid nitrogen elimination. The rate of urea synthesis is a true indicator of whole body protein catabolism. Testosterone reduces protein and nitrogen loss. The effect of testosterone on hepatic urea synthesis in humans has not been studied. OBJECTIVE: To determine whether testosterone reduces hepatic urea production. DESIGN: an open label study Patients and Intervention: Eight hypogonadal men were studied at baseline, and after two weeks of transdermal testosterone replacement (Testogel, 100 mg/day)...
January 25, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28122695/environmental-enteric-dysfunction-is-associated-with-carnitine-deficiency-and-altered-fatty-acid-oxidation
#18
Richard D Semba, Indi Trehan, Ximin Li, Ruin Moaddel, M Isabel Ordiz, Kenneth M Maleta, Klaus Kraemer, Michelle Shardell, Luigi Ferrucci, Mark Manary
BACKGROUND: Environmental enteric dysfunction (EED), a condition characterized by small intestine inflammation and abnormal gut permeability, is widespread in children in developing countries and a major cause of growth failure. The pathophysiology of EED remains poorly understood. METHODS: We measured serum metabolites using liquid chromatography-tandem mass spectrometry in 400 children, aged 12-59months, from rural Malawi. Gut permeability was assessed by the dual-sugar absorption test...
January 18, 2017: EBioMedicine
https://www.readbyqxmd.com/read/28117762/composite-hydrogels-with-engineered-microdomains-for-optical-glucose-sensing-at-low-oxygen-conditions
#19
Lindsey R Bornhoeft, Aniket Biswas, Michael J McShane
There is a growing need for advanced tools that enable frequent monitoring of biomarkers for precision medicine. In this work, we present a composite hydrogel-based system providing real-time optical bioanalyte monitoring. The responsive material, alginate-in-alginate (AnA), is comprised of an alginate hydrogel with embedded bioactive, nanofilm-coated phosphorescent microdomains; palladium tetracarboxyphenylporphyrin serves as an optical indicator, glucose oxidase as a model enzyme, and layer-by-layer deposited polyelectrolyte multilayers (PEMs) as the diffusion barrier...
January 22, 2017: Biosensors
https://www.readbyqxmd.com/read/28111830/mutations-in-the-human-argininosuccinate-synthetase-ass1-gene-impact-on-patients-common-changes-and-structural-considerations
#20
Carmen Diez-Fernandez, Véronique Rüfenacht, Johannes Häberle
Citrullinemia type 1 is an autosomal recessive urea cycle disorder caused by defects in the argininosuccinate synthetase (ASS) enzyme due to mutations in ASS1 gene. An impairment of ASS function can lead to a wide spectrum of phenotypes, from life-threatening neonatal hyperammonemia to a later onset with mild symptoms, and even some asymptomatic patients exhibiting an only biochemical phenotype. The disease is panethnic. In this update, we report 137 mutations (64 of which are novel), consisting of 89 missense mutations, 19 nonsense mutations, 17 mutations that affect splicing and 12 deletions...
January 23, 2017: Human Mutation
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