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https://www.readbyqxmd.com/read/28939132/peak-hyperammonemia-and-atypical-acute-liver-failure-the-eruption-of-an-urea-cycle-disorder-during-hyperemesis-gravidarum
#1
REVIEW
Nicolas Weiss, Fanny Mochel, Marika Rudler, Sophie Demeret, Pascal Lebray, Filomena Conti, Damien Galanaud, Chris Ottolenghi, Jean-Paul Bonnefont, Marc Dommergues, Jacques Bernuau, Dominique Thabut
BACKGROUND: Inborn urea cycle deficiencies are under-recognized metabolic causes of hyperammonaemia in adults. METHODS: A 28-year-old primigravida, 7-week pregnant woman affected by hyperemesis gravidarum developed acute liver injury and then acute liver failure (ALF) in less than 48 hours. RESULTS: Because the patient developed atypical features, especially mildly elevated serum transaminases contrasting with very high blood ammonia levels (281 μmol/l), concomitant with normal serum creatinine, an inborn error of metabolism was suspected...
September 19, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28924015/no-mediates-nitrite-sensing-and-adaptation-and-triggers-a-remodeling-of-lipids
#2
Lina J Dolch, Josselin Lupette, Guillaume Tourcier, Mariette Bedhomme, Séverine Collin, Leonardo Magneschi, Melissa Conte, Khawla Seddiki, Christelle Richard, Erwan Corre, Laurent Fourage, Frédéric Laeuffer, Robert Richards, Michael Reith, Fabrice Rébeillé, Juliette Jouhet, Patrick McGinn, Eric Marechal
Nitric oxide (NO) is an intermediate of the nitrogen cycle, an industrial pollutant and a marker of climate change. NO also acts as a gaseous transmitter in a variety of biological processes. The impact of environmental NO needs to be addressed. In diatoms, a dominant phylum in phytoplankton, NO was reported to mediate programmed cell death (PCD) in response to diatom derived polyunsaturated aldehydes. Here, using the Phaeodactylum Pt1 strain, 2E,4E-decadienal supplied in the micromolar concentration range led to a non-specific cell toxicity...
September 18, 2017: Plant Physiology
https://www.readbyqxmd.com/read/28922379/elevating-serotonin-pre-partum-alters-the-holstein-dairy-cow-hepatic-adaptation-to-lactation
#3
Samantha R Weaver, Allan S Prichard, Noah L Maerz, Austin P Prichard, Elizabeth L Endres, Lorenzo E Hernández-Castellano, Matthew S Akins, Rupert M Bruckmaier, Laura L Hernandez
Serotonin is known to regulate energy and calcium homeostasis in several mammalian species. The objective of this study was to determine if pre-partum infusions of 5-hydroxytryptophan (5-HTP), the immediate precursor to serotonin synthesis, could modulate energy homeostasis at the level of the hepatocyte in post-partum Holstein and Jersey dairy cows. Twelve multiparous Holstein cows and twelve multiparous Jersey cows were intravenously infused daily for approximately 7 d pre-partum with either saline or 1 mg/kg bodyweight of 5-HTP...
2017: PloS One
https://www.readbyqxmd.com/read/28919721/profile-of-sodium-phenylbutyrate-granules-for-the-treatment-of-urea-cycle-disorders-patient-perspectives
#4
REVIEW
Luis Peña-Quintana, Marta Llarena, Desiderio Reyes-Suárez, Luis Aldámiz-Echevarria
Urea-cycle disorders are a group of rare hereditary metabolic diseases characterized by deficiencies of one of the enzymes and transporters involved in the urea cycle, which is necessary for the removal of nitrogen produced from protein breakdown. These hereditary metabolic diseases are characterized by hyperammonemia and life-threatening hyperammonemic crises. Pharmacological treatment of urea-cycle disorders involves alternative nitrogen-scavenging pathways. Sodium benzoate combines with glycine and phenylacetate/phenylbutyrate with glutamine, forming, respectively, hippuric acid and phenylacetylglutamine, which are eliminated in the urine...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28917918/chitosan-composite-hydrogels-reinforced-with-natural-clay-nanotubes
#5
Biao Huang, Mingxian Liu, Changren Zhou
Here, chitosan composites hydrogels were prepared by addition of halloysite nanotubes (HNTs) in the chitosan KOH/LiOH/urea solution. The raw chitosan and chitosan/HNTs composite hydrogels were obtained by heat treatment at 60°C for 8h and then regeneration in ethanol solution. The viscosity of the composite solution is increased with HNTs content. The Fourier transform infrared spectroscopy (FT-IR) shows that the hydrogen bonds interactions exist between the HNTs and the chitosan. X-ray diffraction (XRD) results show that the crystal structure of HNT is not changed in the composite hydrogels...
November 1, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/28916119/safety-and-efficacy-of-glycerol-phenylbutyrate-for-management-of-urea-cycle-disorders-in-patients-aged-2months-to-2years
#6
Susan A Berry, Nicola Longo, George A Diaz, Shawn E McCandless, Wendy E Smith, Cary O Harding, Roberto Zori, Can Ficicioglu, Uta Lichter-Konecki, Beth Robinson, Jerry Vockley
INTRODUCTION: Glycerol phenylbutyrate (GPB) is approved in the US for the management of patients 2months of age and older with urea cycle disorders (UCDs) that cannot be managed with protein restriction and/or amino acid supplementation alone. Limited data exist on the use of nitrogen conjugation agents in very young patients. METHODS: Seventeen patients (15 previously on other nitrogen scavengers) with all types of UCDs aged 2months to 2years were switched to, or started, GPB...
September 8, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28912414/polish-experience-with-liver-transplantation-and-post-transplant-outcomes-in-children-with-urea-cycle-disorders
#7
Edyta Szymańska, Piotr Kaliciński, Joanna Pawłowska, Sylwia Szymańska, Maciej Pronicki, Marek Stefanowicz, Joanna Teisseyre, Dorota Broniszczak, Dariusz Rokicki
BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation...
September 15, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28902935/perconditioning-combined-with-postconditioning-on-kidney-ischemia-and-reperfusion
#8
Felipe Lobato da Silva Costa, Vitor Nagai Yamaki, Renan Kleber Costa Teixeira, Daniel Haber Feijó, André Lopes Valente, Luan Teles Ferreira de Carvalho, Edson Yuzur Yasojima, Marcus Vinicius Henriques Brito
Purpose: To evaluate if combination of perconditioning and postconditioning provides improved renal protection compared to perconditioning alone in a model of renal reperfusion injury. Methods: Thirty rats were assigned into 6 groups: normality; sham; ischemia and reperfusion; postconditioning; perconditioning; perconditioning + postconditioning. Animals were subjected to right nephrectomy and left renal ischemia for 30 minutes. Postconditioning consisted of 3 cycles of 5 min renal perfusion followed by 5 min of renal ischemia after major ischemic period...
August 2017: Acta Cirúrgica Brasileira
https://www.readbyqxmd.com/read/28900784/liver-involvement-in-urea-cycle-disorders-a-review-of-the-literature
#9
REVIEW
Adrien Bigot, Michel C Tchan, Benjamin Thoreau, Hélène Blasco, François Maillot
Urea cycle disorders (UCDs) are inborn errors of metabolism of the nitrogen detoxification pathway and encompass six principal enzymatic deficiencies. The aging of UCD patients leads to a better knowledge of the long-term natural history of the condition and to the reporting of previously unnoticed manifestations. Despite historical evidence of liver involvement in UCDs, little attention has been paid to this organ until recently. Hence, we reviewed the available scientific evidence on acute and chronic liver dysfunction and liver carcinogenesis in UCDs and discuss their pathophysiology...
September 12, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28894242/complementary-acsl-isoforms-contribute-to-a-non-warburg-advantageous-energetic-status-characterizing-invasive-colon-cancer-cells
#10
Ruth Sánchez-Martínez, Silvia Cruz-Gil, María Soledad García-Álvarez, Guillermo Reglero, Ana Ramírez de Molina
Metabolic reprogramming is one of cancer hallmarks. Here, we focus on functional differences and individual contribution of acyl coA synthetases (ACSL) isoforms to the previously described ACSL/stearoyl-CoA desaturase (ACSL1/ACSL4/SCD) metabolic network causing invasion and poor prognosis in colorectal cancer (CRC). ACSL4 fuels proliferation and migration accompanied by a more glycolytic phenotype. Conversely, ACSL1 stimulates invasion displaying a lower basal respiratory rate. Acylcarnitines elevation, polyunsaturated fatty acids (PUFA) lower levels, and monounsaturated fatty acids (MUFA) upregulation characterize the individual overexpression of ACSL1, ACSL4 and SCD, respectively...
September 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28892883/a-case-of-hyperargininaemia-presenting-at-unusually-low-age
#11
Vanita Lal, Daisy Khera, Garima Gupta, Kuldeep Singh, Praveen Sharma
Arginase or ARG1 gene deficiency is a Type V Urea Cycle Disorder (UCD) (catalysing the fifth reaction of urea cycle), associated with hyperammonaemia. Here, we discuss a rare case of a 13-month-old female, having Severe Acute Malnutrition (SAM) and failure to thrive, with serial high plasma ammonia, normal plasma lactate with high arginine and glutamine levels on Amino Acid Assay (AAA) which was performed on 1220 Agilent HPLC. She was admitted for about a month and eventually succumbed to her ailment after a month of discharge...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28888854/quantitation-of-phenylbutyrate-metabolites-by-uplc-ms-ms-demonstrates-inverse-correlation-of-phenylacetate-phenylacetylglutamine-ratio-with-plasma-glutamine-levels
#12
Yi Jiang, Mohammed Almannai, V Reid Sutton, Qin Sun, Sarah H Elsea
Urea cycle disorders (UCDs) are genetic conditions characterized by nitrogen accumulation in the form of ammonia and caused by defects in the enzymes required to convert ammonia to urea for excretion. UCDs include a spectrum of enzyme deficiencies, namely n-acetylglutamate synthase deficiency (NAGS), carbamoyl phosphate synthetase I deficiency (CPS1), ornithine transcarbamylase deficiency (OTC), argininosuccinate lyase deficiency (ASL), citrullinemia type I (ASS1), and argininemia (ARG). Currently, sodium phenylbutyrate and glycerol phenylbutyrate are primary medications used to treat patients with UCDs, and long-term monitoring of these compounds is critical for preventing drug toxic levels...
August 31, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28887792/liver-failure-as-the-presentation-of-ornithine-transcarbamylase-deficiency-in-a-13-month-old-female
#13
Farrah Rajabi, Lance H Rodan, Maureen M Jonas, Janet S Soul, Nicole J Ullrich, Ann Wessel, Susan E Waisbren, Wen-Hann Tan, Gerard T Berry
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle disorder with variable expressivity in heterozygous females. While liver function testing is often abnormal in patients with OTCD, liver failure is uncommon on presentation. A 13-month-old female with no significant past medical history presented with irritability, right arm weakness, and decreased appetite. Initial workup revealed hepatic dysfunction with an INR of 3.4, ammonia level of 75 μmol/L, and abnormal brain MRI with gyral edema with restricted diffusion, and patchy signal abnormality in basal ganglia...
September 9, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28886869/immunohistochemical-study-of-arginases-1-and-2-in-the-olfactory-bulbs-of-the-korean-roe-deer-capreolus-pygargus
#14
Jeongtae Kim, Meejung Ahn, Yuna Choi, Ji-Yeon Hyeon, Taekyun Shin
Arginases are enzymes of the urea cycle that catalyze the hydrolysis of l-arginine to ornithine and urea. The enzymes are core components of the arginine-ornithine-glutamate-γ-amino butyric acid pathway of the central nervous system. In the present study, we immunohistochemically determined the localization of arginase 1 and 2 in the olfactory bulb (OB) of the roe dear (Capreolus pygargus). Reverse transcription PCR revealed that the mRNAs encoding both arginase 1 and 2 were expressed in the OB. Arginase 1 was localized to olfactory nerve axons, calcitonin gene-related peptide-positive mitral/tufted cells (excitatory neurons), and glutamate acid decarboxylase 65/67-immunopositive periglomerular cells of the main olfactory bulb...
September 5, 2017: Acta Histochemica
https://www.readbyqxmd.com/read/28868294/integration-of-proteomics-and-metabolomics-in-exploring-genetic-and-rare-metabolic-diseases
#15
REVIEW
Michele Costanzo, Miriam Zacchia, Giuliana Bruno, Daniela Crisci, Marianna Caterino, Margherita Ruoppolo
BACKGROUND: Inherited metabolic disorders or inborn errors of metabolism are caused by deficiency of enzymatic activities in the catabolism of amino acids, carbohydrates, or lipids. These disorders include aminoacidopathies, urea cycle defects, organic acidemias, defects of oxidation of fatty acids, and lysosomal storage diseases. Inborn errors of metabolism constitute a significant proportion of genetic diseases, particularly in children; however, they are individually rare. Clinical phenotypes are very variable, some of them remain asymptomatic, others manifest metabolic decompensation in neonatal age, and others encompass mental retardation at later age...
July 2017: Kidney Diseases
https://www.readbyqxmd.com/read/28860500/adaptation-of-soil-nitrifiers-to-very-low-nitrogen-level-jeopardizes-the-efficiency-of-chemical-fertilization-in-west-african-moist-savannas
#16
Féline L Assémien, Thomas Pommier, Jean T Gonnety, Jonathan Gervaix, Xavier Le Roux
The moist savanna zone covers 0.5 × 10(6) km(2) in West Africa and is characterized by very low soil N levels limiting primary production, but the ecology of nitrifiers in these (agro)ecosystems is largely unknown. We compared the effects of six agricultural practices on nitrifier activity, abundance and diversity at nine sites in central Ivory Coast. Treatments, including repeated fertilization with ammonium and urea, had no effect on nitrification and crop N status after 3 to 5 crop cycles. Nitrification was actually higher at low than medium ammonium level...
August 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28851525/application-of-a-new-procedure-for-liquid-chromatography-mass-spectrometry-profiling-of-plasma-amino-acid-related-metabolites-and-untargeted-shotgun-proteomics-to-identify-mechanisms-and-biomarkers-of-calcific-aortic-stenosis
#17
Mariola Olkowicz, Janusz Debski, Patrycja Jablonska, Michal Dadlez, Ryszard T Smolenski
Calcific aortic valve stenosis (CAS) increasingly affects our ageing population, but the mechanisms of the disease and its biomarkers are not well established. Recently, plasma amino acid-related metabolite (AA) profiling has attracted attention in studies on pathology and development of biomarkers of cardiovascular diseases, but has not been studied in CAS. To evaluate the potential relationship between CAS and AA metabolome, a new ion-pairing reversed-phase liquid chromatography-tandem mass spectrometry (IP-RPLC-MS/MS) method has been developed and validated for simultaneous determination of 43 AAs in plasma of stenotic patients and age-matched control subjects...
September 29, 2017: Journal of Chromatography. A
https://www.readbyqxmd.com/read/28815739/kernicterus-in-a-boy-with-ornithine-transcarbamylase-deficiency-a-case-report
#18
Eduardo López-Corella, Isabel Ibarra-González, Cynthia Fernández-Lainez, Miguel Á Rodríguez-Weber, Sara Guillén-Lopez, Leticia Belmont-Martínez, David Agüero-Linares, Marcela Vela-Amieva
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle defect associated with severe and usually fatal hyperammonemia. This study describes a patient with early onset lethal OTCD due to a known pathogenic variant (c.298+1G>A), as well as the novel autopsy finding of kernicterus with relatively low blood concentration of unconjugated bilirubin (UCB) (11.55 mg/dL). The patient was a full-term male with a family history of two previous male siblings who died as newborns after acute neurologic deterioration...
August 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28813782/urea-cycle-pathway-targeted-therapeutic-action-of-naringin-against-ammonium-chloride-induced-hyperammonemic-rats
#19
Arumugam Ramakrishnan, Natesan Vijayakumar
Ammonia is a well-known neurotoxin that causes liver disease and urea cycle disorder. Excessive ammonia content in the blood leads to hyperammonemic condition and affects both excitatory and inhibitory neurotransmission including brain edema and coma. Naringin, a plant bioflavonoid present in various citrus fruits and mainly extracted from the grape fruit. This study was designed to assess the protective effect of naringin on ammonium chloride (NH4Cl) induced hyperammonemic rats. Experimental hyperammonemia was induced by intraperitoneal injections (i...
October 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28813487/effect-of-masticatory-stimulation-on-the-quantity-and-quality-of-saliva-and-the-salivary-metabolomic-profile
#20
Nobuyuki Okuma, Makiko Saita, Noriyuki Hoshi, Tomoyoshi Soga, Masaru Tomita, Masahiro Sugimoto, Katsuhiko Kimoto
BACKGROUND: This study characterized the changes in quality and quantity of saliva, and changes in the salivary metabolomic profile, to understand the effects of masticatory stimulation. METHODS: Stimulated and unstimulated saliva samples were collected from 55 subjects and salivary hydrophilic metabolites were comprehensively quantified using capillary electrophoresis-time-of-flight mass spectrometry. RESULTS: In total, 137 metabolites were identified and quantified...
2017: PloS One
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