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Andrés Campolo González, Alex Vargas Díaz, Daniel Fontboté Riesco, Marta Hernández Chávez
INTRODUCTION: Lesch-Nyhan syndrome (LNS) is an inherited recessive X-related disorder caused by the deficiency of the enzyme hypoxanthin-guanine phosphorribosyl transferase (HPRT). Compul sive self-mutilation and dystonia occurs before the first year of age and is expressed by persistent bites on the oral mucosa, lips, tongue, fingers, and shoulders. The dental intervention performed on most of these patients is multiple tooth extraction to prevent serious secondary lesions. OBJECTIVE: To present a clinical case of LNS and describe pediatric dentistry management in patients with self-mutilating behavior...
February 2018: Revista Chilena de Pediatría
T Fukuuchi, N Iyama, N Yamaoka, K Kaneko
Ribonucleotide flavor enhancers such as inosine monophosphate (IMP) and guanosine monophosphate (GMP) provide umami taste, similarly to glutamine. Japanese cuisine frequently uses soup stocks containing these nucleotides to enhance umami. We quantified 18 types of purines (nucleotides, nucleosides, and purine bases) in three soup stocks (chicken, consommé, and dried bonito soup). IMP was the most abundant purine in all umami soup stocks, followed by hypoxanthine, inosine, and GMP. The IMP content of dried bonito soup was the highest of the three soup stocks...
April 13, 2018: Nucleosides, Nucleotides & Nucleic Acids
Masato Furuhashi, Megumi Matsumoto, Marenao Tanaka, Norihito Moniwa, Takayo Murase, Takashi Nakamura, Hirofumi Ohnishi, Shigeyuki Saitoh, Kazuaki Shimamoto, Tetsuji Miura
BACKGROUND: Xanthine oxidoreductase (XOR) is an enzyme that catalyzes the formation of uric acid from hypoxanthine and xanthine, leading to an increase in superoxide and reactive oxygen species. Activation of XOR promotes oxidative stress-related tissue injury. We investigated the associations between metabolic parameters and plasma XOR activity measured by a sensitive and accurate assay using a combination of liquid chromatography and triple quadrupole mass spectrometry to detect [13 C2 ,15 N2 ]-uric acid using [13 C2 ,15 N2 ]-xanthine as a substrate...
April 10, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Angelo D'Alessandro, Julie A Reisz, Rachel Culp-Hill, Herbert Korsten, Robin van Bruggen, Dirk de Korte
BACKGROUND: Over a century of advancements in the field of additive solutions for red blood cell (RBC) storage has made transfusion therapy a safe and effective practice for millions of recipients worldwide. Still, storage in the blood bank results in the progressive accumulation of metabolic alterations, a phenomenon that is mitigated by storage in novel storage additives, such as alkaline additive solutions. While novel alkaline additive formulations have been proposed, no metabolomics characterization has been performed to date...
April 6, 2018: Transfusion
Pollen K Yeung, Chad Purcell, Fatemeh Akhoundi, Remigus U Agu
BACKGROUND: Previous studies have shown that metabolism of adenosine 5'-triphosphate (ATP) in systemic blood is a potential surrogate biomarker for cardiovascular toxicity. OBJECTIVE: To investigate the acute effect of high dose of doxorubicin (DOX) on adenosine and ATP catabolism in systemic blood in vivo. METHOD: Sprague Dawley (SD) rats were each given either 10 mg/kg of DOX (n = 8) or normal saline (1 mL/kg, n = 11) twice daily for 4 doses by subcutaneous (sc) injection...
April 6, 2018: Cardiovascular & Hematological Disorders Drug Targets
Ana Paula de Lima Veeck, Ana Paula Daniel, Bruna Klein, Andréia Quatrin, Ana Paula de Souza Rezer, Liana Guidolin Milani, Carla Cristina Zeppenfeld, Mauro Alves da Cunha, Clarissa Giesel Heldwein, Berta Maria Heinzmann, Thaylise Vey Parodi, Bernardo Baldisserotto, Tatiana Emanuelli
This study evaluated whether the essential oil of Lippia alba (EO) used as a sedative for fish transport would increase the stability of silver catfish during ice storage. Fish were transported (6 h) with water alone (control), 30 or 40 µL/L of EO in water. After transport, fish were slaughtered and stored in ice. Data on mesophilic and psychrotrophic bacteria counts during storage did not support the evidence for the antimicrobial activity of EO. However, fish treated with EO (30 and 40 µL/L) had delayed onset of rigor mortis , delayed increase of pH after 34 days of storage, and delayed peak of hypoxanthine formation and its degradation...
April 2018: Journal of Food Science and Technology
Xiaochang Liu, Zhan Huang, Shiliang Jia, Jingbin Zhang, Kaifeng Li, Yongkang Luo
This study investigated the biochemical changes (proteins degradation, total volatile basic nitrogen, biogenic amines, volatile organic compounds, nucleotides catabolism and related enzymes) of bighead carp samples inoculated with four different bacteria (Shewanella putrefaciens, Aeromonas sobria, Acinetobacter bohemicus, and Pseudomonas helmanticensis) during storage at 4 ± 1 °C. A. sobria exhibited the strongest proteolytic activity. A. sobria, P. helmanticensis, and S. putrefaciens were responsible for putrescine production, whereas S...
July 30, 2018: Food Chemistry
Khalil K Hussain, Mahmood H Akhtar, Moo-Hyun Kim, Dong-Keun Jung, Yoon-Bo Shim
The analytical performance of the multi enzymes loaded single electrode sensor (SES) and dual electrode sensor (DES) was compared for the detection of adenosine and metabolites. The SES was fabricated by covalent binding of tri-enzymes, adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and xanthine oxidase (XO) along with hydrazine (Hyd) onto a functionalized conducting polymer [2,2:5,2-terthiophene-3-(p-benzoic acid)] (pTTBA). The enzyme reaction electrode in DES was fabricated by covalent binding of ADA and PNP onto pTTBA coated on Au nanoparticles...
March 15, 2018: Biosensors & Bioelectronics
Stefano Lazzarano, Marek Kučka, João P L Castro, Ronald Naumann, Paloma Medina, Michael N C Fletcher, Rebecka Wombacher, Joost Gribnau, Tino Hochepied, Marc Van Montagu, Claude Libert, Yingguang Frank Chan
Discovering the genetic changes underlying species differences is a central goal in evolutionary genetics. However, hybrid crosses between species in mammals often suffer from hybrid sterility, greatly complicating genetic mapping of trait variation across species. Here, we describe a simple, robust, and transgene-free technique to generate "in vitro crosses" in hybrid mouse embryonic stem (ES) cells by inducing random mitotic cross-overs with the drug ML216, which inhibits the DNA helicase Bloom syndrome (BLM)...
March 21, 2018: Proceedings of the National Academy of Sciences of the United States of America
Khairunnisa Amreen, Sivakumar Nisha, Annamalai Senthil Kumar
Direct sensing of uric acid (UA) in an undiluted whole blood sample is reported here taking human whole blood as an analyte and a self-supporting electrolyte. Among various solid electrodes (Pt, Au, GCE, and GCE/Nafion) and carbon nanomaterials (carbon nanofibers, graphene oxide, graphite nanopowder, graphitized mesoporous carbon (GMC), single-walled carbon nanotubes, and multiwalled carbon nanotubes) tested, a GMC-modified glassy carbon electrode, designated as GCE/GMC, showed a remarkable response towards direct electrochemical oxidation of blood uric acid at ∼0...
March 16, 2018: Analyst
Dicky Tri Utama, Seung Gyu Lee, Ki Ho Baek, Aera Jang, Jae In Pak, Sung Ki Lee
Objective: This study aimed to observe whether high-pressure processing (HPP) affected aroma development and the degradation rate of umami taste-related ATP breakdown products, specifically inosinic acid in grass-fed beef during vacuum aging. Methods: Strip loin (longissimus lumborum) cuts obtained from six grass-fed Friesian Holstein steers (32 months old) on day 4 post slaughter were vacuum-packed and subjected to pressurization at 300 and 500 MPa for 180 s at 15 ± 2°C...
March 13, 2018: Asian-Australasian Journal of Animal Sciences
Kang-Yi Su, Liang-In Lin, Steven D Goodman, Rong-Syuan Yen, Cho-Yuan Wu, Wei-Chen Chang, Ya-Chien Yang, Wern-Cherng Cheng, Woei-Horng Fang
Deamination of adenine can occur spontaneously under physiological conditions to generate the highly mutagenic lesion, deoxyinosine (hypoxanthine deoxyribonucleotide, dI). In DNA, dI preferably pairs with cytosine rather than thymine and results in A:T to G:C transition mutations after DNA replication. The deamination of adenine is enhanced by ROS from exposure of DNA to ionizing radiation, UV light, nitrous acid, or heat. In Escherichia coli, dI repair is initiated by endonuclease V (endo V; nfi gene product) nicking but a complete repair mechanism has yet to be elucidated...
February 17, 2018: DNA Repair
Marcella Camici, Mercedes Garcia-Gil, Maria Grazia Tozzi
Several physiological functions of adenosine (Ado) appear to be mediated by four G protein-coupled Ado receptors. Ado is produced extracellularly from the catabolism of the excreted ATP, or intracellularly from AMP, and then released through its transporter. High level of intracellular Ado occurs only at low energy charge, as an intermediate of ATP breakdown, leading to hypoxanthine production. AMP, the direct precursor of Ado, is now considered as an important stress signal inside cell triggering metabolic regulation through activation of a specific AMP-dependent protein kinase...
March 9, 2018: International Journal of Molecular Sciences
Shirley Wen-Yu Chiu, Ho-Wen Cheng, Zhi-Xin Chen, Huai-Hsien Wang, Ming-Yu Lai, Juen-Kai Wang, Yuh-Lin Wang
Recently, specific biomarkers in the surface-enhanced Raman scattering (SERS) spectra of bacteria have been successfully exploited for rapid bacterial antibiotic susceptibility testing (AST) - dubbed SERS-AST. The biomolecules responsible for these bacterial SERS biomarkers have been identified as several purine derivative metabolites involved in bacterial purine salvage pathways (W. R. Premasiri, J. C. Lee, A. Sauer-Budge, R. Theberge, C. E. Costello and L. D. Ziegler, Anal. Bioanal. Chem., 2016, 408, 4631)...
March 7, 2018: Physical Chemistry Chemical Physics: PCCP
Sheng Wang, Jianqing Wang, Xiongwen Lv
Investigations of hepatic gene expression are crucial for determining the molecular factors involved in acute alcoholic liver injury. The results of liver molecular investigations may reveal etiologically important genomic alterations. Therefore, it is necessary to normalize gene expression data to identify stable genes, which may be used as a reference under different experimental conditions. The aim of the present study was to apply reverse transcription‑quantitative polymerase chain reaction analysis and use analysis software to investigate the expression stability of candidate reference genes in hepatic tissues from mice with acute alcoholic liver injury...
March 1, 2018: International Journal of Molecular Medicine
Habib Mosbah, Hassiba Chahdoura, Jannet Kammoun, Malek Besbes Hlila, Hanen Louati, Saoussen Hammami, Guido Flamini, Lotfi Achour, Boulbaba Selmi
BACKGROUND: α-glucosidase is a therapeutic target for diabetes mellitus (DM) and α-glucosidase inhibitors play a vital role in the treatments for the disease. Furthermore, xanthine oxidase (XO) is a key enzyme that catalyzes hypoxanthine and xanthine to uric acid which at high levels can lead to hyperuricemia which is an important cause of gout. Pancreatic lipase (PL) secreted into the duodenum plays a key role in the digestion and absorption of fats. For its importance in lipid digestion, PL represents an attractive target for obesity prevention...
March 5, 2018: BMC Complementary and Alternative Medicine
Halima W M Al Balushi, David C Rees, John N Brewin, Anke Hannemann, John S Gibson
Red cells from patients with sickle cell anemia (SCA) are under greater oxidative challenge than those from normal individuals. We postulated that oxidants generated by xanthine oxidase (XO) and hypoxanthine (HO) contribute to the pathogenesis of SCA through altering solute permeability. Sickling, activities of the main red cell dehydration pathways (Psickle , Gardos channel, and KCl cotransporter [KCC]), and cell volume were measured at 100, 30, and 0 mmHg O2 , together with deoxygenation-induced nonelectrolyte hemolysis...
March 2018: Physiological Reports
Hideki Makinoshima, Shigeki Umemura, Ayako Suzuki, Hiroki Nakanishi, Ami Maruyama, Hibiki Udagawa, Sachiyo Mimaki, Shingo Matsumoto, Seiji Niho, Genichiro Ishii, Masahiro Tsuboi, Atsushi Ochiai, Hiroyasu Esumi, Takehiko Sasaki, Koichi Goto, Katsuya Tsuchihara
Comprehensive genomic analysis has revealed that the PI3K/AKT/mTOR pathway is a feasible therapeutic target in small-cell lung carcinoma (SCLC). However, biomarkers to identify patients likely to benefit from inhibitors of this pathway have not been identified. Here we show that metabolic features determine sensitivity to the PI3K/mTOR dual inhibitor gedatolisib in SCLC cells. Substantial phosphatidyl lipid analysis revealed that a specific phosphatidylinositol (3,4,5)-trisphosphate (PIP3) subspecies lipid product: PIP3 (38:4) is predictive in assessing sensitivity to PI3K/mTOR dual inhibitor...
February 28, 2018: Cancer Research
J Scott Lee, Ruth X Wang, Erica E Alexeev, Jordi M Lanis, Kayla D Battista, Louise E Glover, Sean P Colgan
Intestinal epithelial cells form a selectively permeable barrier to protect colon tissues from luminal microbiota and antigens, and to mediate nutrient, fluid, and waste flux in the intestinal tract. Dysregulation of the epithelial cell barrier coincides with profound shifts in metabolic energy, especially in the colon, which exists in an energetically depleting state of physiological hypoxia. However, studies that systematically examine energy flux and adenylate metabolism during intestinal epithelial barrier development and restoration after disruption are lacking...
February 27, 2018: Journal of Biological Chemistry
Joshua D Bryant, Shannon R Sweeney, Enrique Sentandreu, Minhye Shin, Helene Ipas, Blerta Xhemalce, Jessica Momb, Stefano Tiziani, Dean R Appling
One-carbon (1C) metabolism is a universal folate-dependent pathway essential for de novo purine and thymidylate synthesis, amino acid interconversion, universal methyl-donor production, and regeneration of redox cofactors. Homozygous deletion of the 1C pathway gene Mthfd1l encoding methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like, which catalyzes mitochondrial formate production from 10-formyltetrahydrofolate, results in 100% penetrant embryonic neural tube defects (NTDs), underscoring the central role of mitochondrially derived formate in embryonic development and providing a mechanistic link between folate and NTDs...
February 26, 2018: Journal of Biological Chemistry
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