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red cell membrane defect

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https://www.readbyqxmd.com/read/28164768/protective-effect-of-aspirin-against-oligomeric-a%C3%AE-42-induced-mitochondrial-alterations-and-neurotoxicity-in-differentiated-ec-p19-neuronal-cells
#1
Hamendra Singh Parmar, Zbynek Houdek, Martin Pesta, Vaclava Cerna, Pavel Dvorak, Jiri Hatina
Amyloid-beta (Aβ) induced mitochondrial dysfunction is one of the major causes of neuronal toxicity in Alzheimer's disease. Many recent reports suggest involvement of mitochondrial alterations through intracellular accumulation of oligomeric Aβ. These mitochondrial alterations include increased reactive oxygen species (ROS), mt-DNA depletion, decreased oxidative phosphorylation and ATP production, membrane depolarization, reduced number of mitochondria etc. These all defects cumulatively caused neural toxicity and alterations in cellular energy homeostasis...
February 2, 2017: Current Alzheimer Research
https://www.readbyqxmd.com/read/28116040/mitotempo-prevents-oxalate-induced-injury-in-nrk-52e-cells-via-inhibiting-mitochondrial-dysfunction-and-modulating-oxidative-stress
#2
Jiaqiao Zhang, Qing Wang, Chuou Xu, Yuchao Lu, Henglong Hu, Baolong Qin, Yufeng Wang, Deng He, Cong Li, Xiao Yu, Shaogang Wang, Jihong Liu
As one of the major risks for urolithiasis, hyperoxaluria can be caused by genetic defect or dietary intake. And high oxalate induced renal epithelial cells injury is related to oxidative stress and mitochondrial dysfunction. Here, we investigated whether MitoTEMPO, a mitochondria-targeted antioxidant, could protect against oxalate mediated injury in NRK-52E cells via inhibiting mitochondrial dysfunction and modulating oxidative stress. MitoSOX Red was used to determine mitochondrial ROS (mtROS) production...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28095524/combination-of-collagen-barrier-membrane-with-enamel-matrix-derivative-liquid-improves-osteoblast-adhesion-and-differentiation
#3
Richard J Miron, Masako Fujioka-Kobayashi, Daniel Buser, Yufeng Zhang, Dieter D Bosshardt, Anton Sculean
PURPOSE: Collagen barrier membranes were first introduced to regenerative periodontal and oral surgery to prevent fast ingrowing soft tissues (ie, epithelium and connective tissue) into the defect space. More recent attempts have aimed at combining collagen membranes with various biologics/growth factors to speed up the healing process and improve the quality of regenerated tissues. Recently, a new formulation of enamel matrix derivative in a liquid carrier system (Osteogain) has demonstrated improved physico-chemical properties for the adsorption of enamel matrix derivative to facilitate protein adsorption to biomaterials...
January 2017: International Journal of Oral & Maxillofacial Implants
https://www.readbyqxmd.com/read/28079132/critical-role-for-cafen1-and-cafen12-of-candida-albicans-in-cell-wall-integrity-and-biofilm-formation
#4
Md Alfatah, Vinay K Bari, Anubhav S Nahar, Swati Bijlani, K Ganesan
Sphingolipids are involved in several cellular functions, including maintenance of cell wall integrity. To gain insight into the role of individual genes of sphingolipid biosynthetic pathway, we have screened Saccharomyces cerevisiae strains deleted in these genes for sensitivity to cell wall perturbing agents calcofluor white and congo red. Only deletants of FEN1 and SUR4 genes were found to be sensitive to both these agents. Candida albicans strains deleted in their orthologs, CaFEN1 and CaFEN12, respectively, also showed comparable phenotypes, and a strain deleted for both these genes was extremely sensitive to cell wall perturbing agents...
January 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28067475/the-chaperonin-tric-forms-an-oligomeric-complex-in-the-malaria-parasite-cytosol
#5
Natalie J Spillman, Josh R Beck, Suresh M Ganesan, Jacquin C Niles, Daniel E Goldberg
The malaria parasite exports numerous proteins into its host red blood cell (RBC). The trafficking of these exported effectors is complex. Proteins are first routed through the secretory system, into the parasitophorous vacuole (PV), a membranous compartment enclosing the parasite. Proteins are then translocated across the PV membrane in a process requiring ATP and unfolding. Once in the RBC compartment the exported proteins are then refolded and further trafficked to their final localizations. Chaperones are important in the unfolding and refolding processes...
January 9, 2017: Cellular Microbiology
https://www.readbyqxmd.com/read/28044234/development-of-a-regulatable-expression-system-for-the-functional-study-of-vibrio-vulnificus-essential-genes
#6
Yan-Jiao Zhang, Guozhong Chen, Huiyuan Lin, Pan Wang, Baozhi Kuang, Jing Liu, Shiyong Chen
We developed a regulatable gene expression system for Vibrio vulnificus, which contains a lacIq-pTrc cassette. Monomeric red fluorescence protein (mRFP) was used as a reporter to test this system. The results showed that this system tightly controlled the expression of mRFP without leaky expression and was suitable for the controlled expression of genes encoding recombinant proteins in V. vulnificus. To demonstrate the utility of this system, a dominant negative form of V. vulnificus VVMO6_RS04990, a homolog of Escherichia coli LolD that is essential in lipoprotein transport and membrane biogenesis, was inducibly expressed...
January 2, 2017: Antonie Van Leeuwenhoek
https://www.readbyqxmd.com/read/27932475/intercalated-cell-depletion-and-vacuolar-h-atpase-mistargeting-in-an-ae1-r607h-knockin-model
#7
Rizwan Mumtaz, Francesco Trepiccione, J Christopher Hennings, Antje K Huebner, Bettina Serbin, Nicolas Picard, A K M Shahid Ullah, Teodor G Păunescu, Diane E Capen, Rawad M Lashhab, Isabelle Mouro-Chanteloup, Seth L Alper, Carsten A Wagner, Emmanuelle Cordat, Dennis Brown, Dominique Eladari, Christian A Hübner
Distal nephron acid secretion is mediated by highly specialized type A intercalated cells (A-ICs), which contain vacuolar H(+)-ATPase (V-type ATPase)-rich vesicles that fuse with the apical plasma membrane on demand. Intracellular bicarbonate generated by luminal H(+) secretion is removed by the basolateral anion-exchanger AE1. Chronically reduced renal acid excretion in distal renal tubular acidosis (dRTA) may lead to nephrocalcinosis and renal failure. Studies in MDCK monolayers led to the proposal of a dominant-negative trafficking mechanism to explain AE1-associated dominant dRTA...
December 8, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27792725/md-dpd-multiscale-framework-for-predicting-morphology-and-stresses-of-red-blood-cells-in-health-and-disease
#8
Hung-Yu Chang, Xuejin Li, He Li, George Em Karniadakis
Healthy red blood cells (RBCs) have remarkable deformability, squeezing through narrow capillaries as small as 3 microns in diameter without any damage. However, in many hematological disorders the spectrin network and lipid bilayer of diseased RBCs may be significantly altered, leading to impaired functionality including loss of deformability. We employ a two-component whole-cell multiscale model to quantify the biomechanical characteristics of the healthy and diseased RBCs, including Plasmodium falciparum-infected RBCs (Pf-RBCs) and defective RBCs in hereditary disorders, such as spherocytosis and elliptocytosis...
October 2016: PLoS Computational Biology
https://www.readbyqxmd.com/read/27756835/new-insights-on-hereditary-erythrocyte-membrane-defects
#9
REVIEW
Immacolata Andolfo, Roberta Russo, Antonella Gambale, Achille Iolascon
After the first proposed model of the red blood cell membrane skeleton 36 years ago, several additional proteins have been discovered during the intervening years, and their relationship with the pathogenesis of the related disorders have been somewhat defined. The knowledge of erythrocyte membrane structure is important because it represents the model for spectrin-based membrane skeletons in all cells and because defects in its structure underlie multiple hemolytic anemias. This review summarizes the main features of erythrocyte membrane disorders, dividing them into structural and altered permeability defects, focusing particularly on the most recent advances...
November 2016: Haematologica
https://www.readbyqxmd.com/read/27742708/a-new-molecular-link-between-defective-autophagy-and-erythroid-abnormalities-in-chorea-acanthocytosis
#10
Francesca Lupo, Elena Tibaldi, Alessandro Matte, Alok K Sharma, Anna Maria Brunati, Seth L Alper, Carlo Zancanaro, Donatella Benati, Angela Siciliano, Mariarita Bertoldi, Francesca Zonta, Alexander Storch, Ruth H Walker, Adrian Danek, Benedikt Bader, Andreas Hermann, Lucia De Franceschi
Chorea-acanthocytosis is one of the hereditary neurodegenerative disorders known as the neuroacanthocytoses. Chorea-acanthocytosis is characterized by circulating acanthocytes deficient in chorein, a protein of unknown function. We report here for the first time that chorea-acanthocytosis red cells are characterized by impaired autophagy, with cytoplasmic accumulation of active Lyn and of autophagy-related proteins Ulk1 and Atg7. In chorea-acanthocytosis erythrocytes, active Lyn is sequestered by HSP90-70 to form high-molecular-weight complexes that stabilize and protect Lyn from its proteasomal degradation, contributing to toxic Lyn accumulation...
December 22, 2016: Blood
https://www.readbyqxmd.com/read/27725587/genetic-diagnosis-for-congenital-hemolytic-anemia
#11
Shouichi Ohga
Congenital hemolytic anemia is a group of monogenic diseases presenting with anemia due to increased destruction of circulating erythrocytes. The etiology of inherited anemia accounts for germline mutations of the responsible genes coding for the structural components of erythrocytes and extra-erythrocytes. The erythrocyte abnormalities are classified into three major disorders of red cell membrane defects, hemoglobinopathies, and red cell enzymopathies. The extra-erythrocyte abnormalities, typified by consumption coagulopathy and intravascular hemolysis, include Upshaw-Schulman syndrome and atypical hemolytic uremic syndrome...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27716630/evaluation-of-3d-printed-pcl-plga-%C3%AE-tcp-versus-collagen-membranes-for-guided-bone-regeneration-in-a-beagle-implant-model
#12
J-Y Won, C-Y Park, J-H Bae, G Ahn, C Kim, D-H Lim, D-W Cho, W-S Yun, J-H Shim, J-B Huh
Here, we compared 3D-printed polycaprolactone/poly(lactic-co-glycolic acid)/β-tricalcium phosphate (PCL/PLGA/β-TCP) membranes with the widely used collagen membranes for guided bone regeneration (GBR) in beagle implant models. For mechanical property comparison in dry and wet conditions and cytocompatibility determination, we analyzed the rate and pattern of cell proliferation of seeded fibroblasts and preosteoblasts using the cell counting kit-8 assay and scanning electron microscopy. Osteogenic differentiation was verified using alizarin red S staining...
October 7, 2016: Biomedical Materials
https://www.readbyqxmd.com/read/27667160/genotype-phenotype-correlations-in-hereditary-elliptocytosis-and-hereditary-pyropoikilocytosis
#13
Omar Niss, Satheesh Chonat, Neha Dagaonkar, Marya O Almansoori, Karol Kerr, Zora R Rogers, Patrick T McGann, Maa-Ohui Quarmyne, Mary Risinger, Kejian Zhang, Theodosia A Kalfa
Hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP) are heterogeneous red blood cell (RBC) membrane disorders that result from mutations in the genes encoding α-spectrin (SPTA1), β-spectrin (SPTB), or protein 4.1R (EPB41). The resulting defects alter the horizontal cytoskeletal associations and affect RBC membrane stability and deformability causing shortened RBC survival. The clinical diagnosis of HE and HPP relies on identifying characteristic RBC morphology on peripheral blood smear and specific membrane biomechanical properties using osmotic gradient ektacytometry...
October 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27593084/fatty-acid-related-modulations-of-membrane-fluidity-in-cells-detection-and-implications
#14
G Maulucci, O Cohen, B Daniel, A Sansone, P I Petropoulou, S Filou, A Spyridonidis, G Pani, M De Spirito, C Chatgilialoglu, C Ferreri, K E Kypreos, S Sasson
Metabolic homeostasis of fatty acids is complex and well-regulated in all organisms. The biosynthesis of saturated fatty acids (SFA) in mammals provides substrates for β-oxidation and ATP production. Monounsaturated fatty acids (MUFA) are products of desaturases that introduce a methylene group in cis geometry in SFA. Polyunsaturated fatty acids (n-6 and n-3 PUFA) are products of elongation and desaturation of the essential linoleic acid and α-linolenic acid, respectively. The liver processes dietary fatty acids and exports them in lipoproteins for distribution and storage in peripheral tissues...
November 2016: Free Radical Research
https://www.readbyqxmd.com/read/27557951/topological-structures-and-membrane-nanostructures-of-erythrocytes-after-splenectomy-in-hereditary-spherocytosis-patients-via-atomic-force-microscopy
#15
Ying Li, Liyuan Lu, Juan Li
Hereditary spherocytosis is an inherited red blood cell membrane disorder resulting from mutations of genes encoding erythrocyte membrane and cytoskeletal proteins. Few equipments can observe the structural characteristics of hereditary spherocytosis directly expect for atomic force microscopy In our study, we proved atomic force microscopy is a powerful and sensitive instrument to describe the characteristics of hereditary spherocytosis. Erythrocytes from hereditary spherocytosis patients were small spheroidal, lacking a well-organized lattice on the cell membrane, with smaller cell surface particles and had reduced valley to peak distance and average cell membrane roughness vs...
September 2016: Cell Biochemistry and Biophysics
https://www.readbyqxmd.com/read/27539300/fluorescent-protein-vectors-for-pancreatic-islet-cell-identification-in-live-cell-imaging
#16
Hongyan Shuai, Yunjian Xu, Qian Yu, Erik Gylfe, Anders Tengholm
The islets of Langerhans contain different types of endocrine cells, which are crucial for glucose homeostasis. β- and α-cells that release insulin and glucagon, respectively, are most abundant, whereas somatostatin-producing δ-cells and particularly pancreatic polypeptide-releasing PP-cells are more scarce. Studies of islet cell function are hampered by difficulties to identify the different cell types, especially in live-cell imaging experiments when immunostaining is unsuitable. The aim of the present study was to create a set of vectors for fluorescent protein expression with cell-type-specific promoters and evaluate their applicability in functional islet imaging...
October 2016: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/27471141/analysis-of-a-cohort-of-101-cdaii-patients-description-of-24-new-molecular-variants-and-genotype-phenotype-correlations
#17
Paola Bianchi, Klaus Schwarz, Josef Högel, Elisa Fermo, Cristina Vercellati, Regine Grosse, Richard van Wijk, Rob van Zwieten, Wilma Barcellini, Alberto Zanella, Hermann Heimpel
Congenital dyserythropoietic anaemia type II (CDAII) is a rare autosomal recessive disease characterized by ineffective erythropoiesis, haemolysis, erythroblast morphological abnormalities, hypoglycosylation of some red blood cell membrane proteins, particularly band 3, and mutations in the SEC23B gene. We report the analysis of 101 patients from 91 families with a median follow-up of 23 years (range 0-65); 68 patients are newly reported. Clinical and haematological parameters were separately analysed in early infancy and thereafter, when feasible...
November 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27427187/detection-of-new-pathogenic-mutations-in-patients-with-congenital-haemolytic-anaemia-using-next-generation-sequencing
#18
R Del Orbe Barreto, B Arrizabalaga, A B De la Hoz, Á García-Orad, M I Tejada, J C Garcia-Ruiz, T Fidalgo, C Bento, L Manco, M L Ribeiro
INTRODUCTION: Congenital haemolytic anaemia (CHA) refers to a group of genetically heterogeneous disorders, mainly caused by changes in genes encoding globin chains, cytoskeletal proteins and red cell enzymes, in which accurate diagnosis can be challenging with conventional techniques. METHODS: To set-up a comprehensive assay for detecting mutations that could improve aetiological diagnosis, we designed a custom panel for sequencing coding regions from 40 genes known to be involved in the pathogenesis of CHA, using the Ion Torrent(™) (Thermo Fisher Scientific, S...
December 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27367668/mechanisms-underlying-activation-of-%C3%AE-%C3%A2-adrenergic-receptor-induced-trafficking-of-aqp5-in-rat-parotid-acinar-cells-under-isotonic-or-hypotonic-conditions
#19
Aneta M Bragiel, Di Wang, Tomasz D Pieczonka, Masayuki Shono, Yasuko Ishikawa
Defective cellular trafficking of aquaporin-5 (AQP5) to the apical plasma membrane (APM) in salivary glands is associated with the loss of salivary fluid secretion. To examine mechanisms of α₁-adrenoceptor (AR)-induced trafficking of AQP5, immunoconfocal microscopy and Western blot analysis were used to analyze AQP5 localization in parotid tissues stimulated with phenylephrine under different osmolality. Phenylephrine-induced trafficking of AQP5 to the APM and lateral plasma membrane (LPM) was mediated via the α1A-AR subtype, but not the α1B- and α1D-AR subtypes...
June 28, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27353637/advances-in-understanding-the-pathogenesis-of-the-red-cell-volume-disorders
#20
REVIEW
Catherine Badens, Hélène Guizouarn
Genetic defects of erythrocyte transport proteins cause disorders of red blood cell volume that are characterized by abnormal permeability to the cations Na(+) and K(+) and, consequently, by changes in red cell hydration. Clinically, these disorders are associated with chronic haemolytic anaemia of variable severity and significant co-morbidities, such as iron overload. This review provides an overview of recent insights into the molecular basis of this group of rare anaemias involving cation channels and transporters dysfunction...
September 2016: British Journal of Haematology
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