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https://www.readbyqxmd.com/read/29896245/changes-of-lysosomal-membrane-permeabilization-and-lipid-metabolism-in-sidt2-deficient-mice
#1
Yu Meng, Lizhuo Wang, Liefeng Ling
The SID1 transmembrane family member 2 (sidt2) deficient mouse model was used to investigate the function of sidt2 in lysosomal membrane permeabilization and lipid metabolism of liver tissue. The mouse model was established by Cre/LoxP technology. Enzymatic methods were used to analyze the sidt2-/- mouse serum lipids, aspartate transaminase, alanine transaminase and serum bilirubin, compared with sidt2+/+ mice. Defective lipid metabolism and damaged liver functions were observed in the sidt2-/- mice. By using hematoxylin and eosin and Oil Red O staining, changes of morphology were observed in sidt2-/- mice with optical microscopy...
July 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29878199/integrating-glycomics-and-genomics-uncovers-slc10a7-as-essential-factor-for-bone-mineralization-by-regulating-post-golgi-protein-transport-and-glycosylation
#2
Angel Ashikov, Nurulamin Abu Bakar, Xiao-Yan Wen, Marco Niemeijer, Glentino Rodrigues Pinto Osorio, Koroboshka Brand-Arzamendi, Linda Hasadsri, Hana Hansikova, Kimiyo Raymond, Dorothée Vicogne, Marleen E H Simon, Rolph Pfundt, Sharita Timal, Roel Beumers, Christophe Biot, Roel Smeets, Marjan Kersten, Karin Huijben, Peter Ta Linders, Geert van den Bogaart, Sacha A F T van Hijum, Richard Rodenburg, Lambertus P van den Heuvel, Francjan van Spronsen, Tomas Honzik, Francois Foulquier, Monique van Scherpenzeel, Dirk J Lefeber
Genomics methodologies have significantly improved elucidation of Mendelian disorders. The combination with high-throughput functional-omics technologies potentiates the identification and confirmation of causative genetic variants, especially in singleton families of recessive inheritance. In a cohort of 99 individuals with abnormal Golgi glycosylation, 47 of which being unsolved, glycomics profiling was performed of total plasma glycoproteins. Combination with whole-exome sequencing in 31 cases revealed a known genetic defect in 15 individuals...
June 5, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29755372/use-of-laser-assisted-optical-rotational-cell-analyzer-lorrca-maxsis-in-the-diagnosis-of-rbc-membrane-disorders-enzyme-defects-and-congenital-dyserythropoietic-anemias-a-monocentric-study-on-202-patients
#3
Anna Zaninoni, Elisa Fermo, Cristina Vercellati, Dario Consonni, Anna P Marcello, Alberto Zanella, Agostino Cortelezzi, Wilma Barcellini, Paola Bianchi
Chronic hemolytic anemias are a group of heterogeneous diseases mainly due to abnormalities of red cell (RBC) membrane and metabolism. The more common RBC membrane disorders, classified on the basis of blood smear morphology, are hereditary spherocytosis (HS), elliptocytosis, and hereditary stomatocytoses (HSt). Among RBC enzymopathies, the most frequent is pyruvate kinase (PK) deficiency, followed by glucose-6-phosphate isomerase, pyrimidine 5' nucleotidase P5'N, and other rare enzymes defects. Because of the rarity and heterogeneity of these diseases, diagnosis may be often challenging despite the availability of a variety of laboratory tests...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29676105/-effects-of-tongbi-capsule-on-joint-lesions-in-rabbits-with-rheumatoid-arthritis
#4
Qing-Yong Cao, Pei-Yuan Wang, Qiao-Yun Wang, Feng Zhao, Ke-Zhan Wang, Jun-Qi Liu, Tong-Shen Liu, Yuan-Fang Wang, Yong-Jun Liu
The purpose of this experiment is to observe the effects of Tongbi capsule on joint lesions in rabbit with rheumatoid arthritis induced by ovalbumin and explore the mechanism in order to provide reference for clinical application of Tongbi capsule. Rheumatoid arthritis in rabbits was induced by subcutaneous injection of emulsions of ovalbumin and Freund's complete adjuvant and intra articular injection of ovalbumin. After successful modeling, 30 New Zealand rabbits with arthritis were randomly divided into model control group, the high, medium and low dose groups of Tongbi capsule (90, 45, 22...
March 2018: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29611201/a-practical-toxicity-bioassay-for-vicine-and-convicine-levels-in-faba-bean-vicia-faba
#5
Fitsum Getachew, Albert Vandenberg, Judit Smits
BACKGROUND: Faba bean (Vicia faba) vicine and convicine (V-C) aglycones (divicine and isouramil respectively) provoke an acute hemolytic anemia called favism in individuals with a glucose-6-phosphate dehydrogenase (G6PD) enzyme defect in their red blood cells. Geneticists/plant breeders are working with faba bean to decrease V-C levels to improve public acceptance of this high-protein pulse crop. Here, we present a fast and simple ex vivo in vitro bioassay for V-C toxicity testing of faba bean or faba bean food products...
April 2, 2018: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/29475008/comparison-of-the-effects-of-rosuvastatin-monotherapy-and-atorvastatin-ezetimibe-combined-therapy-on-the-structure-of-erythrocyte-membranes-in-patients-with-coronary-artery-disease
#6
Małgorzata Olszewska-Banaszczyk, Paulina Jackowska, Paulina Gorzelak-Pabiś, Edyta Pytel, Maria Koter-Michalak, Marlena Broncel
BACKGROUND: Abnormalities in the physical properties of the red blood cells (RBCs) membranes may underlie the defects that are strongly linked to cardiovascular diseases (CVD). The aim of the study was to compare the effects of two therapies of equal hipolipemic efficacy on the erythrocyte membrane fluidity, concentration of membrane cholesterol, lipids peroxidation and RBCs distribution witdh in patients with CVD. METHODS: The study included 44 patients with angiographic evidence of CVD, who despite previous 6-month hypolipemic therapy, did not achieve the concentration of LDL-C <70mg/dl...
April 2018: Pharmacological Reports: PR
https://www.readbyqxmd.com/read/29447902/the-erythrocyte-osmotic-resistance-test-as-screening-tool-for-cholesterol-related-lysosomal-storage-diseases
#7
Laura López de Frutos, Jorge J Cebolla, Pilar Irún, Ralf Köhler, Pilar Giraldo
BACKGROUND: Erythrocyte volume regulation and membrane elasticity are essential for adaptation to osmotic and mechanical stress, and life span. Here, we evaluated whether defective cholesterol trafficking caused by the rare lysosomal storages diseases (LSDs), Niemann-Pick type C (NPC) and Lysosomal acid lipase (LAL) deficiency (LALD) impairs these properties. Moreover, we tested whether measurements of cholesterol membrane content and osmotic resistance serve as a screening test for these LSDs...
May 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29407748/characterization-of-a-bioactive-jagged1-coated-polycaprolactone-based-membrane-for-guided-tissue-regeneration
#8
Nunthawan Nowwarote, Phunphimp Chanjavanakul, Pattanit Kongdecha, Panipuk Clayhan, Sujin Chumprasert, Jeeranan Manokawinchoke, Hiroshi Egusa, Prasit Pavasant, Thanaphum Osathanon
OBJECTIVE: The aim of the present study was to develop a Jagged1-coated polycaprolactone (PCL) membrane and to evaluate the response of human periodontal ligament cells (hPDL) on this membrane in vitro. METHODS: Membranes were prepared from PCL and PCL-incorporated hydroxyapatite (PCL/HA). The membranes' surface roughness, surface wettability, and mechanical properties were examined. An indirect affinity immobilization technique was used to coat the membranes with Jagged1...
April 2018: Archives of Oral Biology
https://www.readbyqxmd.com/read/29364234/measuring-deformability-and-red-cell-heterogeneity-in-blood-by-ektacytometry
#9
Nermi L Parrow, Pierre-Christian Violet, Hongbin Tu, James Nichols, Corinne A Pittman, Courtney Fitzhugh, Robert E Fleming, Narla Mohandas, John F Tisdale, Mark Levine
Decreased red cell deformability is characteristic of several disorders. In some cases, the extent of defective deformability can predict severity of disease or occurrence of serious complications. Ektacytometry uses laser diffraction viscometry to measure the deformability of red blood cells subject to either increasing shear stress or an osmotic gradient at a constant value of applied shear stress. However, direct deformability measurements are difficult to interpret when measuring heterogenous blood that is characterized by the presence of both rigid and deformable red cells...
January 12, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29311237/evaluation-of-cpxra-as-a-therapeutic-target-for-uropathogenic-escherichia-coli-infections
#10
Lana Dbeibo, Julia J van Rensburg, Sara N Smith, Kate R Fortney, Dharanesh Gangaiah, Hongyu Gao, Juan Marzoa, Yunlong Liu, Harry L T Mobley, Stanley M Spinola
CpxRA is an envelope stress response system found in all members of the family Enterobacteriaceae ; CpxA has kinase activity for CpxR and phosphatase activity for phospho-CpxR, a transcription factor. CpxR also accepts phosphate groups from acetyl phosphate, a glucose metabolite. Activation of CpxR increases the transcription of genes encoding membrane repair and downregulates virulence determinants. We hypothesized that activation of CpxR could serve as an antimicrobial/antivirulence strategy and discovered compounds that activate CpxR in Escherichia coli by inhibiting CpxA phosphatase activity...
March 2018: Infection and Immunity
https://www.readbyqxmd.com/read/29296844/potential-impact-of-complement-regulator-deficiencies-on-hemolytic-reactions-due-to-minor-abo-mismatched-transfusions
#11
Priyanka Pandey, Waseem Q Anani, Jerome L Gottschall, Gregory A Denomme
Minor ABO-mismatched transfusions are a common occurrence, although infrequent transfusion reactions occur. We sought to investigate the regulation of complement C3 activation induced by anti-A. In vitro complement C3 activation was observed with 10 of 30 group O samples and correlated with immunoglobulin M (IgM) anti-A titers. We developed an in vitro paroxysmal nocturnal hemoglobinuria (PNH) model of hemolysis in which group A1 red blood cells (RBCs) were chemically treated with 2-aminoethylisothiouronium (AET) to alter regulators of complement C3 activation...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29090523/digital-microscopy-as-a-screening-tool-for-the-diagnosis-of-hereditary-hemolytic-anemia
#12
R Huisjes, W W van Solinge, M D Levin, R van Wijk, J A Riedl
INTRODUCTION: Evaluation of red blood cell (RBC) morphology is an important first step in the differential diagnosis of hereditary hemolytic anemia. It is, however, labor intensive, expensive, and prone to subjectivity. To improve and standardize the analysis of RBC morphology as a screening tool in the diagnosis of hereditary hemolytic anemia, we studied its automated analysis by digital microscopy (DM). METHODS: Blood from 90 patients with hereditary hemolytic anemia and 32 normal control subjects was analyzed by the CellaVision DM96 Digital Microscope...
April 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28729432/tropomodulin-1-controls-erythroblast-enucleation-via-regulation-of-f-actin-in-the-enucleosome
#13
Roberta B Nowak, Julien Papoin, David S Gokhin, Carla Casu, Stefano Rivella, Jeffrey M Lipton, Lionel Blanc, Velia M Fowler
Biogenesis of mammalian red blood cells requires nuclear expulsion by orthochromatic erythoblasts late in terminal differentiation (enucleation), but the mechanism is largely unexplained. Here, we employed high-resolution confocal microscopy to analyze nuclear morphology and F-actin rearrangements during the initiation, progression, and completion of mouse and human erythroblast enucleation in vivo. Mouse erythroblast nuclei acquire a dumbbell-shaped morphology during enucleation, whereas human bone marrow erythroblast nuclei unexpectedly retain their spherical morphology...
August 31, 2017: Blood
https://www.readbyqxmd.com/read/28721813/mechanisms-involved-in-the-increased-hemolysis-in-the-fetus-and-newborn
#14
REVIEW
Bracci Rodolfo, Perrone Serafina, Buonocore Giuseppe
The suicidal death of erythrocytes should be considered a possible cause of hemolysis and plasma bilirubin overload when there is no evidence of an immune-mediated hemolytic anemia, no consumptive red blood cell disorder, no morphologic or laboratory data to suggest a problem of the red cell membrane, and no evidence of a quantitative or qualitative defect in hemoglobin synthesis. In neonatal period, xenobiotics, cytokines, osmotic shock, energy depletion, oxidative stress, and variation of temperature may induce an alteration of balance between damaging and protecting factors which can be followed by red cell death...
2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28683142/the-plasmodium-falciparum-pseudoprotease-sera5-regulates-the-kinetics-and-efficiency-of-malaria-parasite-egress-from-host-erythrocytes
#15
Christine R Collins, Fiona Hackett, Jonathan Atid, Michele Ser Ying Tan, Michael J Blackman
Egress of the malaria parasite Plasmodium falciparum from its host red blood cell is a rapid, highly regulated event that is essential for maintenance and completion of the parasite life cycle. Egress is protease-dependent and is temporally associated with extensive proteolytic modification of parasite proteins, including a family of papain-like proteins called SERA that are expressed in the parasite parasitophorous vacuole. Previous work has shown that the most abundant SERA, SERA5, plays an important but non-enzymatic role in asexual blood stages...
July 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28680334/expression-of-cd55-cd59-and-cd35-on-red-blood-cells-of-%C3%AE-thalassaemia-patients
#16
Ayşegül Uǧur Kurtoǧllu, Belkls Koçtekin, Erdal Kurtoǧlu, Mustafa Yildiz, Selen Bozkurt
AIM OF THE STUDY: β-thalassaemia (β-Thal) is considered a severe, progressive haemolytic anaemia, which needs regular blood transfusions for life expectancy. Complement-mediated erythrocyte destruction can cause both intravascular and extravascular haemolysis. Complement regulatory proteins protect cells from such effects of the complement system. We aimed to perform quantitative analysis of membrane-bound complement regulators, CD55 (decay accelerating factor - DAF), CD35 (complement receptor type 1 - CR1), and CD59 (membrane attack complex inhibitory factor - MACIF) on peripheral red blood cells by flow cytometry...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28646781/%C3%AE-potential-evidences-silanol-heterogeneity-induced-by-metal-contaminants-at-the-quartz-surface-implications-in-membrane-damage
#17
Cristina Pavan, Francesco Turci, Maura Tomatis, Mara Ghiazza, Dominique Lison, Bice Fubini
Among the physico-chemical features responsible for the so-called "variability of quartz hazard", a key role has been assigned to the silica surface charge, evaluated by means of ζ potential measurement. The ζ potential of silica describes the protonation state of silanols which, in turn, determine interactions with cell membranes. To gain a molecular understanding of the role of silanols in silica pathogenicity, we conducted a systematic investigation of the variation of the ζ potential as a function of pH (ζ plot titration curve) on a large set of respirable quartz particles with different levels of metal contaminants...
September 1, 2017: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/28614812/the-discovery-of-water-channels-aquaporins
#18
REVIEW
Dennis Brown
The movement of water into and out of cells is a fundamental biological process that is essential for life. Such water movement not only regulates the activity of individual cells but also is responsible for the functioning of many organ systems and for maintaining whole body water balance. It had long been suspected that water movement across biological cell membranes was in some way enhanced or facilitated by pores or channels, but the search to identify these channels was long and tedious. As is often the case in science, the secret of the water channel was eventually discovered by chance in 1992 by Peter Agre and his colleagues at the Johns Hopkins University in Baltimore, who were working on red blood cell membrane proteins...
2017: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/28550188/recommendations-regarding-splenectomy-in-hereditary-hemolytic-anemias
#19
Achille Iolascon, Immacolata Andolfo, Wilma Barcellini, Francesco Corcione, Loïc Garçon, Lucia De Franceschi, Claudio Pignata, Giovanna Graziadei, Dagmar Pospisilova, David C Rees, Mariane de Montalembert, Stefano Rivella, Antonella Gambale, Roberta Russo, Leticia Ribeiro, Jules Vives-Corrons, Patricia Aguilar Martinez, Antonis Kattamis, Beatrice Gulbis, Maria Domenica Cappellini, Irene Roberts, Hannah Tamary
Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications...
August 2017: Haematologica
https://www.readbyqxmd.com/read/28280328/gambogic-acid-loaded-biomimetic-nanoparticles-in-colorectal-cancer-treatment
#20
Zhen Zhang, Hanqing Qian, Mi Yang, Rutian Li, Jing Hu, Li Li, Lixia Yu, Baorui Liu, Xiaoping Qian
Gambogic acid (GA) is expected to be a potential new antitumor drug, but its poor aqueous solubility and inevitable side effects limit its clinical application. Despite these inhe rent defects, various nanocarriers can be used to promote the solubility and tumor targeting of GA, improving antitumor efficiency. In addition, a cell membrane-coated nanoparticle platform that was reported recently, unites the customizability and flexibility of a synthetic copolymer, as well as the functionality and complexity of natural membrane, and is a new synthetic biomimetic nanocarrier with improved stability and biocompatibility...
2017: International Journal of Nanomedicine
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