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Blood substitute

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https://www.readbyqxmd.com/read/28803808/two-novel-mutations-p-ser160pro-and-p-arg472cys-causing-glucose-6-phosphate-isomerase-deficiency-are-associated-with-erythroid-dysplasia-and-inappropriately-suppressed-hepcidin
#1
Renata Mojzikova, Pavla Koralkova, Dusan Holub, Zuzana Saxova, Dagmar Pospisilova, Daniela Prochazkova, Petr Dzubak, Monika Horvathova, Vladimir Divoky
Glucose-6-phosphate isomerase (GPI) deficiency, a genetic disorder responsible for chronic nonspherocytic hemolytic anemia, is the second most common red blood cell glycolytic enzymopathy. We report three patients from two unrelated families of Czech and Slovak origin with macrocytic hemolytic anemia due to GPI deficiency. The first patient had 15% of residual GPI activity resulting from two new heterozygous missense mutations c.478T>C and c.1414C>T leading to substitutions p.(Ser160Pro) and p.(Arg472Cys)...
April 14, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28802692/de-novo-transcriptome-assembly-and-rna-seq-expression-analysis-in-blood-from-beluga-whales-of-bristol-bay-ak
#2
Jeanine S Morey, Kathy A Burek Huntington, Michelle Campbell, Tonya M Clauss, Caroline E Goertz, Roderick C Hobbs, Denise Lunardi, Amanda J Moors, Marion G Neely, Lori H Schwacke, Frances M Van Dolah
Assessing the health of marine mammal sentinel species is crucial to understanding the impacts of environmental perturbations on marine ecosystems and human health. In Arctic regions, beluga whales, Delphinapterus leucas, are upper level predators that may serve as a sentinel species, potentially forecasting impacts on human health. While gene expression profiling from blood transcriptomes has widely been used to assess health status and environmental exposures in human and veterinary medicine, its use in wildlife has been limited due to the lack of available genomes and baseline data...
August 9, 2017: Marine Genomics
https://www.readbyqxmd.com/read/28802207/the-effect-of-aluminium-and-sodium-impurities-on-the-in-vitro-toxicity-and-pro-inflammatory-potential-of-cristobalite
#3
C Nattrass, C J Horwell, D E Damby, D Brown, V Stone
BACKGROUND: Exposure to crystalline silica (SiO2), in the form of quartz, tridymite or cristobalite, can cause respiratory diseases, such as silicosis. However, the observed toxicity and pathogenicity of crystalline silica is highly variable. This has been attributed to a number of inherent and external factors, including the presence of impurities. In cristobalite-rich dusts, substitutions of aluminium (Al) for silicon (Si) in the cristobalite structure, and impurities occluding the silica surface, have been hypothesised to decrease its toxicity...
August 9, 2017: Environmental Research
https://www.readbyqxmd.com/read/28798999/influence-of-immunostimulant-polysaccharides-nucleic-acids-and-bacillus-strains-on-the-innate-immune-and-acute-stress-response-in-turbots-scophthalmus-maximus-fed-soy-bean-and-wheat-based-diets
#4
V I Fuchs, J Schmidt, M J Slater, B H Buck, D Steinhagen
Immunostimulants are widely applied in aquaculture practice and may have beneficial effects on the immune system and physical functions allowing higher tolerance to stress. In the current study, the impact of four (i-iv) dietary active ingredients on the immune and stress response of turbot was examined in two experiments (I and II). A basal low fish meal (FM; 32%) diet was formulated and supplemented with (i) yeast β-glucan and mannan oligosaccharide (GM), (ii) alginic acid (AC), (iii) yeast nucleotides and RNA (NR), or (iv) Bacillus strains (BS)...
August 10, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/28797049/effect-of-sodium-bicarbonate-on-prolonged-running-performance-a-randomized-double-blind-cross-over-study
#5
Tanja Freis, Anne Hecksteden, Ulf Such, Tim Meyer
BACKGROUND: The ability to sustain intense exercise seems to be partially limited by the body's capability to counteract decreases in both intra- and extracellular pH. While the influence of an enhanced buffering capacity via sodium bicarbonate (BICA) on short-term, high-intensity exercise performance has been repeatedly investigated, studies on prolonged endurance performances are comparatively rare, especially for running. The aim of the following study was to assess the ergogenic effects of an oral BICA substitution upon exhaustive intensive endurance running performance...
2017: PloS One
https://www.readbyqxmd.com/read/28794333/development-of-time-course-oxygen-binding-analysis-for-hemoglobin-based-oxygen-carriers
#6
Yukihiro Okamoto, Shigenori Sugisaki, Keishi Suga, Hiroshi Umakoshi
Developing blood substitutes is in urgent demand for chronic blood shortage all over the world. In this connection, the oxygen binding behavior of hemoglobin-based oxygen carriers (HBOCs) is one of the most important characteristics. However, present methods available for estimating oxygen binding behavior have need of expensive apparatus, and also are not suitable for high-throughput and the time-course analysis. To overcome these problems, we proposed a simple analysis method for the time-course oxygen binding behavior of HBOCs, which employs a general UV-Vis microplate reader and a common reagent, sodium dithionite, as a reductant for HBOCs and an oxygen scavenger...
2017: Analytical Sciences: the International Journal of the Japan Society for Analytical Chemistry
https://www.readbyqxmd.com/read/28792545/contribution-of-independent-and-pleiotropic-genetic-effects-in-the-metabolic-syndrome-in-a-hypertensive-rat
#7
Man Chun John Ma, Janette M Pettus, Jessica A Jakoubek, Matthew G Traxler, Karen C Clark, Amanda K Mennie, Anne E Kwitek
Hypertension is a major risk factor for cardiovascular disease, Type 2 diabetes, and end organ failure, and is often found concomitant with disorders characteristic of the Metabolic Syndrome (MetS), including obesity, dyslipidemia, and insulin resistance. While the associated features often occur together, the pathway(s) or mechanism(s) linking hypertension in MetS are not well understood. Previous work determined that genetic variation on rat chromosome 17 (RNO17) contributes to several MetS-defining traits (including hypertension, obesity, and dyslipidemia) in the Lyon Hypertensive (LH) rat, a genetically determined MetS model...
2017: PloS One
https://www.readbyqxmd.com/read/28789699/oral-pyridoxine-can-substitute-for-intravenous-pyridoxine-in-managing-patients-with-severe-poisoning-with-isoniazid-and-rifampicin-fixed-dose-combination-tablets-a-case-report
#8
M D S A Dilrukshi, C A P Ratnayake, C A Gnanathasan
BACKGROUND: Fixed drug combination of isoniazid and rifampicin is a rare cause of poisoning even in endemic countries for tuberculosis infection. Severe poisoning can cause severe morbidity and mortality if not treated promptly. Though intravenous pyridoxine is the preferred antidote for severe standard isoniazid poisoning it is not freely available even in best of care centers. We describe a case of severe poisoning with fixed drug combination of isoniazid and rifampicin successfully managed with oral pyridoxine at national hospital of Sri Lanka...
August 8, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28782047/characterization-of-protein-z-dependent-protease-inhibitor-antithrombin-chimeras-provides-insight-into-the-serpin-specificity-of-coagulation-proteases
#9
Likui Yang, Alireza R Rezaie
Protein Z (PZ)-dependent protease inhibitor (ZPI) and antithrombin (AT) are two physiological serpin inhibitors involved in the regulation of proteolytic activities of the blood coagulation cascade. ZPI has restricted protease specificity capable of inhibiting factors Xa (FXa) and XIa (FXIa) but exhibiting no reactivity with other coagulation proteases. Unlike ZPI, AT is a general inhibitor of all coagulation proteases and the only physiological inhibitor of factor IXa (FIXa). To understand the molecular determinants of protease specificity of the two serpins, we engineered two ZPI mutants in which the P12-P3' residues of the reactive center loop of ZPI were replaced with either P12-P3' or P12-P7' residues of AT (ZPI-AT(P12-P3') and ZPI-AT(P12-P7'))...
July 31, 2017: ACS Omega
https://www.readbyqxmd.com/read/28777868/-study-of-467c-t-and-745c-t-variants-of-alpha-1-3-n-acetylgalactoseaminotransferase-gene
#10
Xu Zhang, Jianping Li
OBJECTIVE: To explore the effect of alpha-1,3-N-acetylgalactosaminyltransferase gene variants on A antigen expression in a family where a member was suspected for a rare A3 subtype of the ABO variant. METHODS: Serological assay was carried out to determine the ABO blood group of the proband and his family members. To determine the haploid of the alpha-1,3-N-acetylgalactosaminyltransferase gene of the proband, DNA was extracted and genotyped with sequence-specific primer PCR (PCR-SSP) followed by direct sequencing and cloning of exons 6 and 7 of the ABO locus...
August 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28772236/a-novel-glucagon-like-peptide-1-glucagon-receptor-dual-agonist-exhibits-weight-lowering-and-diabetes-protective-effects
#11
Jie Zhou, Xingguang Cai, Xun Huang, Yuxuan Dai, Lidan Sun, Bo Zhang, Bo Yang, Haiyan Lin, Wenlong Huang, Hai Qian
Glucagon has plenty of effects via a specific glucagon receptor(GCGR) like elevating the blood glucose, improving fatty acids metabolism, energy expenditure and increasing lipolysis in adipose tissue. The most important role of glucagon is to regulate the blood glucose, but the emergent possibilities of hyperglycaemia is exist. Glucagon could also slightly activate glucagon-like peptide-1 receptor(GLP-1R), which lead to blood glucose lowering effect. This study aims to erase the likelihood of hyperglycaemia and to remain the inherent catabolic effects through improving GLP-1R activation and deteriorating GCGR activation so as to lower the bodyweight and show diabetes-protective effects...
July 24, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28771262/commercially-available-gluten-free-pastas-elevate-postprandial-glycemia-in-comparison-to-conventional-wheat-pasta-in-healthy-adults-a-double-blind-randomized-crossover-trial
#12
C S Johnston, D Snyder, C Smith
Given the popularity of gluten-free diets, research regarding the health implications of gluten-free (GF) products is necessary. This study compared the postprandial glycemic responses to three GF pastas commonly available in the U.S. market to that of wheat pasta in healthy adults. Thirteen healthy non-smoking men and women from a university campus population were enrolled in this randomized 4 × 4 block crossover study and completed all four treatments. Participants followed a standardized diet and activity protocol the day prior to testing, and one week separated testing periods...
August 3, 2017: Food & Function
https://www.readbyqxmd.com/read/28763452/pathogen-reduction-through-additive-free-short-wave-uv-light-irradiation-retains-the-optimal-efficacy-of-human-platelet-lysate-for-the-expansion-of-human-bone-marrow-mesenchymal-stem-cells
#13
Sabrina Viau, Lucie Chabrand, Sandy Eap, Judith Lorant, Karl Rouger, Francis Goudaliez, Chryslain Sumian, Bruno Delorme
BACKGROUND: We recently developed and characterized a standardized and clinical grade human Platelet Lysate (hPL) that constitutes an advantageous substitute for fetal bovine serum (FBS) for human mesenchymal stem cell (hMSC) expansion required in cell therapy procedures, avoiding xenogenic risks (virological and immunological) and ethical issues. Because of the progressive use of pathogen-reduced (PR) labile blood components, and the requirement of ensuring the viral safety of raw materials for cell therapy products, we evaluated the impact of the novel procedure known as THERAFLEX UV-Platelets for pathogen reduction on hPL quality (growth factors content) and efficacy (as a medium supplement for hMSC expansion)...
2017: PloS One
https://www.readbyqxmd.com/read/28762037/genetic-polymorphism-of-growth-differentiation-factor-9-gdf9-gene-related-to-fecundity-in-two-egyptian-sheep-breeds
#14
Zaki A El Fiky, Gamal M Hassan, Mohamed I Nassar
PURPOSE: This study explores polymorphisms in the growth differentiation factor 9 (GDF9) gene (exon 1) with respect to fertility in Egyptian sheep. METHODS: Blood samples were collected, and genomic DNA was extracted from 24 Saidi and 13 Ossimi ewes. A 710 bp portion of the GDF9 gene, was amplified using specific primers, and the sequence was analyzed to clarify the phylogenetic relationship of Egyptian breed sheep. In addition, the PCR-RFLP method using Pst1 or Msp1 restriction enzymes was used to mask polymorphisms of partial exon 1 of GDF9 gene to establish molecular markers for twinning...
July 31, 2017: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/28757744/hla-haplotype-in-association-with-the-low-incidence-c-07-66-allele-found-by-case-analysis-of-taiwanese-and-mainland-chinese-individuals
#15
Kuo-Liang Yang, Zheng-Zhong Zheng
OBJECTIVES: HLA-C*07:66 is a low-incidence HLA-C allele. The aim of the study is to report the Taiwanese and mainland Chinese ethnicities of individuals with C*07:66, together with its uniqueness and polymorphism. MATERIALS AND METHODS: A sequence-based typing method was employed to confirm this low-incidence allele. Polymerase chain reaction was performed to amplify exons 2, 3, and 4 of the HLA-A, HLA-B, and HLA-C loci and exon 2 of the HLA-DRB1 and HLA-DQB1 loci using group-specific primer sets...
October 2016: Ci Ji Yi Xue za Zhi, Tzu-chi Medical Journal
https://www.readbyqxmd.com/read/28757411/increased-ophthalmic-acid-production-is-supported-by-amino-acid-catabolism-under-fasting-conditions-in-mice
#16
Sho Kobayashi, Jaeyong Lee, Toshifumi Takao, Junichi Fujii
Glutathione (GSH) plays pivotal roles in antioxidation and detoxification. The transsulfuration pathway, in conjunction with methionine metabolism, produces equimolar amounts of cysteine (Cys) and 2-oxobutyric acid (2OB). The resulting 2OB is then converted into 2-aminobutyric acid (2AB) by a transaminase and is utilized as a substitute for Cys by the GSH-synthesizing machinery to produce ophthalmic acid (OPT). By establishing a method for simultaneously measuring Cys, GSH, and OPT by liquid chromatography-mass spectrometry, we found that fasting causes an elevation in OPT levels in the liver and blood plasma, even though the levels of Cys and GSH are decreased...
July 28, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28754700/segregation-of-mitochondrial-dna-mutations-in-the-human-placenta-implication-for-prenatal-diagnosis-of-mtdna-disorders
#17
Pauline Vachin, Elodie Adda-Herzog, Gihad Chalouhi, Caroline Elie, Marlène Rio, Sophie Rondeau, Nadine Gigarel, Fabienne Jabot Hanin, Sophie Monnot, Roxana Borghese, Joana Bengoa, Yves Ville, Agnes Rotig, Arnold Munnich, Jean-Paul Bonnefont, Julie Steffann
BACKGROUND: Mitochondrial DNA (mtDNA) disorders have a high clinical variability, mainly explained by variation of the mutant load across tissues. The high recurrence risk of these serious diseases commonly results in requests from at-risk couples for prenatal diagnosis (PND), based on determination of the mutant load on a chorionic villous sample (CVS). Such procedures are hampered by the lack of data regarding mtDNA segregation in the placenta.The objectives of this report were to determine whether mutant loads (1) are homogeneously distributed across the whole placentas, (2) correlate with those in amniocytes and cord blood cells and (3) correlate with the mtDNA copy number...
July 28, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28753979/lack-of-association-between-hepatitis-c-virus-core-gene-variation-70-91aa-and-insulin-resistance
#18
Letícia de Paula Scalioni, Allan Peres da Silva, Juliana Custódio Miguel, Márcia Paschoal do Espírito Santo, Vanessa Alves Marques, Carlos Eduardo Brandão-Mello, Cristiane Alves Villela-Nogueira, Lia Laura Lewis-Ximenez, Elisabeth Lampe, Livia Melo Villar
The role of hepatitis C virus (HCV) in insulin resistance (IR) is not fully understood. The aim of this study was to determine the impact of amino acid (aa) substitutions in the core region of HCV according to IR and to identify clinical and laboratory associations. Ninety-two treatment-naive HCV patients were recruited to determine laboratory data and blood cell count. IR was determined using Homeostasis Model Assessment (HOMA) index where IR was defined as HOMA ≥2. HCV RNA load and genotype were determined by Abbott Real time HCV...
July 21, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28753684/erratum-unexpected-serious-cardiac-arrhythmias-in-the-setting-of-loperamide-abuse
#19
Somwail Rasla, Parag Parikh, Peter Hoffmeister, Amy St Amand, Marina K Garas, Amr El Meligy, Taro Minami, Nishant R Shah
Loperamide (Imodium) is a non-prescription opioid receptor agonist available over-the-counter for the treatment of diarrhea. When ingested in excessive doses, loperamide can penetrate the blood-brain barrier and is reported to produce euphoria, central nervous system and respiratory depression, and cardiotoxicity. There is an emerging trend in its use among drug abusers for its euphoric effects or for self-treatment of opioid withdrawal. We report a case of ventricular dysrhythmias associated with loperamide abuse in a 28-year-old man who substituted loperamide for the opioids that he used to abuse...
August 1, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28749473/slc39a8-deficiency-biochemical-correction-and-major-clinical-improvement-by-manganese-therapy
#20
Julien H Park, Max Hogrebe, Manfred Fobker, Renate Brackmann, Barbara Fiedler, Janine Reunert, Stephan Rust, Konstantinos Tsiakas, René Santer, Marianne Grüneberg, Thorsten Marquardt
PurposeSLC39A8 deficiency is a severe inborn error of metabolism that is caused by impaired function of manganese metabolism in humans. Mutations in SLC39A8 lead to impaired function of the manganese transporter ZIP8 and thus manganese deficiency. Due to the important role of Mn(2+) as a cofactor for a variety of enzymes, the resulting phenotype is complex and severe. The manganese-dependence of β-1,4-galactosyltransferases leads to secondary hypoglycosylation, making SLC39A8 deficiency both a disorder of trace element metabolism and a congenital disorder of glycosylation...
July 27, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
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