Search results for "ANEM"

Evaluation of Clotting Factor Concentrates for Treatment of Thrombotic Thrombocytopenic Purpura.

2010

Abstract Abstract 3678 Introduction: Thrombotic thrombocytopenic purpura (TTP) is characterized by microthrombi, hemolytic anemia as well as thrombocytopenia. These symptoms are caused by a decreased activity of the protease ADAMTS13 which cleaves the von Willebrand Factor (VWF), due to mutation of the ADAMTS13-gene or autoantibodies. At the moment, the only available immediate therapy is plasmapheresis with Fresh Frozen Plasma (FFP) which may induce side effects. Therefore an alternative therapy might be the treatment with clotting factor concentrates. Methods: 40 plasma samples were tested, consisting of FFP and solvent/detergent treated plasma, four batches of each blood group; VWF/VIII …

The Mucus of Actinia equina (Anthozoa, Cnidaria): An Unexplored Resource for Potential Applicative Purposes

2015

The mucus produced by many marine organisms is a complex mixture of proteins and polysaccharides forming a weak watery gel. It is essential for vital processes including locomotion, navigation, structural support, heterotrophic feeding and defence against a multitude of environmental stresses, predators, parasites, and pathogens. In the present study we focused on mucus produced by a benthic cnidarian, the sea anemone Actinia equina (Linnaeus, 1758) for preventing burial by excess sedimentation and for protection. We investigated some of the physico-chemical properties of this matrix such as viscosity, osmolarity, electrical conductivity, protein, carbohydrate, and total lipid contents. Som…

Note critique : A World of Difference ? Comparing Learners Across Europe ? OSBORN M . ; BROADFOOT P . ; MC NESS E . ; PLANEL C . ; RAVN B . & TRIGGS …

2005

Compromised repair of radiation-induced DNA double-strand breaks in Fanconi anemia fibroblasts in G2

2020

Fanconi anemia (FA) is a rare chromosomal instability syndrome with various clinical features and high cancer incidence. Despite being a DNA repair disorder syndrome and a frequently observed clinical hypersensitivity of FA patients towards ionizing radiation, the experimental evidence regarding the efficiency of radiation-induced DNA double-strand break (DSB) repair in FA is very controversial. Here, we performed a thorough analysis of the repair of radiation-induced DSBs in G1 and G2 in FA fibroblasts of complementation groups A, C, D1 (BRCA2), D2, E, F, G and P (SLX4) in comparison to normal human lung and skin fibroblasts. γH2AX, 53BP1, or RPA foci quantification after X-irradiation was…

Lentiviral-Mediated Gene Therapy in Fanconi Anemia-A Mice Reveals Long-Term Engraftment and Continuous Turnover of Corrected HSCs

2015

International audience; Fanconi anemia is a DNA repair-deficiency syndrome mainly characterized by cancer predisposition and bone marrow failure. Trying to restore the hematopoietic function in these patients, lentiviral vector-mediated gene therapy trials have recently been proposed. However, because no insertional oncogenesis studies have been conducted so far in DNA repair-deficiency syndromes such as Fanconi anemia, we have carried out a genome-wide screening of lentiviral insertion sites after the gene correction of Fanca-/- hematopoietic stem cells (HSCs), using LAM-PCR and 454-pyrosequencing. Our studies first demonstrated that transduction of Fanca-/- HSCs with a lentiviral vector d…

Oxidative stress in Fanconi anaemia: from cells and molecules towards prospects in clinical management.

2011

Abstract Fanconi anaemia (FA) is a genetic disease featuring bone marrow failure, proneness to malignancies, and chromosomal instability. A line of studies has related FA to oxidative stress (OS). This review attempts to evaluate the evidence for FA-associated redox abnormalities in the literature from 1981 to 2010. Among 2170 journal articles on FA evaluated, 162 related FA with OS. Early studies reported excess oxygen toxicity in FA cells that accumulated oxidative DNA damage. Prooxidant states were found in white blood cells and body fluids from FA patients as excess luminol-dependent chemiluminescence, 8-hydroxy-deoxyguanosine, reduced glutathione/oxidized glutathione imbalance, and tum…

Impact of a Three Amino Acid Deletion in the CH2 Domain of Murine IgG1 on Fc-Associated Effector Functions

2008

Abstract Four murine IgG subclasses display markedly different Fc-associated effector functions because of their differential binding to three activating IgG Fc receptors (FcγRI, FcγRIII, and FcγRIV) and C1q. Previous analysis of IgG subclass switch variants of 34-3C anti-RBC monoclonal autoantibodies revealed that the IgG1 subclass, which binds only to FcγRIII and fails to activate complement, displayed the poorest pathogenic potential. This could be related to the presence of a three amino acid deletion at positions 233–235 in the CH2 domain uniquely found in this subclass. To address this question, IgG1 insertion and IgG2b deletion mutants at positions 233–235 of 34-3C anti-RBC Abs were …

Estudio epidemiológico de las parasitosis intestinales detectadas en la población infantil de Ruanda (África Central)

2016

La presente Tesis Doctoral tiene por objetivo conocer el estado del parasitismo intestinal y su correlación con diferentes factores intrínsecos y extrínsecos en la población infantil ruandesa. Para ello, se ha estudiado un total de 674 escolares (330 niños y 344 niñas) del Colegio Nemba I (Nemba, Gakenke, Província Norte de Ruanda), con edades comprendidas entre los 6 y 18 años de edad. Se ha detectado un espectro parasitario de 17 especies (10 de protozoos y 7 de helmintos), con una prevalencia total de parasitación del 94,9% (94,5% para protozoos y 18,1% para helmintos). Las especies más prevalentes fueron: Endolimax nana (91,1%), Blastocystis hominis (89,9%) y Entamoeba coli (57,2%). Se …

Remote sensing of volcanic CO2, HF, HCl, SO2, and BrO in the downwind plume of Mt. Etna

2017

Remote sensing of the gaseous composition of non-eruptive, passively degassing volcanic plumes can be a tool to gain insight into volcano interior processes. Here, we report on a field study in September 2015 that demonstrates the feasibility of remotely measuring the volcanic enhancements of carbon dioxide (CO2), hydrogen fluoride (HF), hydrogen chloride (HCl), sulfur dioxide (SO2), and bromine monoxide (BrO) in the downwind plume of Mt. Etna using portable and rugged spectroscopic instrumentation. To this end, we operated the Fourier transform spectrometer EM27/SUN for the shortwave-infrared (SWIR) spectral range together with a co-mounted UV spectrometer on a mobile platform in direct-su…

MORPHODYNAMIC PROCESSES DOWNSTREAM OF MAN-MADE STRUCTURAL INTERVENTIONS: experimental investigation of the role of turbulent flow structures in the p…

2012

Abstract Experimental program was conducted to analyze scour – caused by a horizontal jet downstream of a rigid bed – and the flow velocity and turbulence characteristics within the scour hole. The experimental program was motivated by the fact that the knowledge of flow field, including turbulent characteristics, is fundamental to correctly simulate the bed-load transport and the temporal evolution of the scour hole and, thus, to define protective measures of scouring. In this paper some results of experiments – carried out to understand the role of flow turbulence in the prediction of scouring process and sediment movement – are reported. The analysis is conducted on the basis of detailed…