6,709 research outputs found

    Effects of tributyltin chloride on cybrids with or without an ATP synthase pathologic mutation

    Get PDF
    Background: The oxidative phosphorylation system (OXPHOS) includes nuclear chromosome (nDNA)– and mitochondrial DNA (mtDNA)–encoded polypeptides. Many rare OXPHOS disorders, such as striatal necrosis syndromes, are caused by genetic mutations. Despite important advances in sequencing procedures, causative mutations remain undetected in some patients. It is possible that etiologic factors, such as environmental toxins, are the cause of these cases. Indeed, the inhibition of a particular enzyme by a poison could imitate the biochemical effects of pathological mutations in that enzyme. Moreover, environmental factors can modify the penetrance or expressivity of pathological mutations. Objectives: We studied the interaction between mitochondrially encoded ATP synthase 6 (p.MT-ATP6) subunit and an environmental exposure that may contribute phenotypic differences between healthy individuals and patients suffering from striatal necrosis syndromes or other mitochondriopathies. Methods: We analyzed the effects of the ATP synthase inhibitor tributyltin chloride (TBTC), a widely distributed environmental factor that contaminates human food and water, on transmitochondrial cell lines with or without an ATP synthase mutation that causes striatal necrosis syndrome. Doses were selected based on TBTC concentrations previously reported in human whole blood samples. Results: TBTC modified the phenotypic effects caused by a pathological mtDNA mutation. Interestingly, wild-type cells treated with this xenobiotic showed similar bioenergetics when compared with the untreated mutated cells. Conclusions: In addition to the known genetic causes, our findings suggest that environmental exposure to TBTC might contribute to the etiology of striatal necrosis syndromes

    Effect of palladium on gold in core-shell catalyst for electrooxidation of ethanol in alkaline medium

    Get PDF
    In this paper the effect of small amounts of palladium deposited on gold nanoparticles supported on Vulcan XC-72 carbon (core-shell structure denoted Au@Pd/C) is studied. Different nominal atomic compositional ratios of Au@Pdx maintaining fixed gold nuclei and varying the amount of palladium (x = 0.10; 0.80 and 1.60) were synthesized via seed growth method for the ethanol oxidation reaction in alkaline medium. UV–Vis spectrometric, X-ray powder diffraction, X-ray energy dispersive spectroscopy, transmission electron microscopy and electrochemical measurements were performed for the characterization of these catalysts. Electrocatalytic activity toward ethanol oxidation on Au@Pd/C catalysts were investigated by cyclic voltammetry and chronoamperometry showed that [email protected]/C electrocatalyst has the highest current density and low onset potential for ethanol oxidation reaction in alkaline medium. In-situ Fourier transform infrared spectroscopy measurements demonstrated that acetate is the main product of ethanol oxidation and CO2 can be slightest observed, the latter could be visualized in greater quantity on catalyst [email protected]/C catalyst

    Changes in Lolium perenne L. rhizosphere microbiome during phytoremediation of Cd- and Hg-contaminated soils

    Get PDF
    The contamination of soil and water by metals such as mercury (Hg) and cadmium (Cd) has been increasing in recent years, because of anthropogenic activities such as mining and agriculture, respectively. In this work, the changes in the rhizosphere microbiome of Lolium perenne L. during the phytoremediation of soils contaminated with Hg and Cd were evaluated. For this, two soil types were sampled, one inoculated with mycorrhizae and one without. The soils were contaminated with Hg and Cd, and L. perenne seeds were sown and harvested after 30 days. To assess changes in the microbiome, DNA isolation tests were performed, for which samples were subjected to two-step PCR amplification with specific 16S rDNA V3-V4 primers (337F and 805R). With mycorrhizae, changes had been found in the absorption processes of metals and a new distribution. While with respect to microorganisms, families such as the Enterobacteriaceae have been shown to have biosorption and efflux effects on metals such as Hg and Cd. Mycorrhizae then improve the efficiency of removal and allow the plant to better distribute the absorbed concentrations. Overall, L. perenne is a species with a high potential for phytoremediation of Cd- and Hg-contaminated soils in the tropics. Inoculation with mycorrhizae modifies the phytoremediation mechanisms of the plant and the composition of microorganisms in the rhizosphere. Mycorrhizal inoculation and changes in the microbiome were associated with increased plant tolerance to Cd and Hg. Microorganism-assisted phytoremediation is an appropriate alternative for L. perenne

    Higher platelet cytochrome oxidase specific activity in surviving than in non-surviving septic patients

    Get PDF
    Introduction: In a previous study with 96 septic patients, we found that circulating platelets in 6-months surviving septic patients showed higher activity and quantity of cytochrome c oxidase (COX) normalized by citrate synthase (CS) activity at moment of severe sepsis diagnosis than non-surviving septic patients. The objective of this study was to estimate whether COX specific activity during the first week predicts 1-month sepsis survival in a larger cohort of patients.Methods: Using a prospective, multicenter, observational study carried out in six Spanish intensive care units with 198 severe septic patients, we determined COX activity per proteins (COXact/Prot) in circulating platelets at day 1, 4 and 8 of the severe sepsis diagnosis. Endpoints were 1-month and 6-months mortality.Results: Survivor patients (n = 130) showed higher COXact/Prot (P 0.30 mOD/min/mg at day 1 (P = 0.002), 4 (P = 0.006) and 8 (P = 0.02) was associated independently with 1-month mortality. Area under the curve of COXact/Prot at day 1, 4 and 8 to predict 30-day survival were 0.70 (95% CI = 0.63-0.76; P < 0.001), 0.71 (95% CI = 0.64-0.77; P < 0.001) and 0.71 (95% CI = 0.64-0.78; P < 0.001), respectively.Conclusions: The new findings of our study, to our knowledge the largest series reporting data about mitochondrial function during follow-up in septic patients, were that septic patients that survive 1-month have a higher platelet cytochrome oxidase activity at moment of sepsis diagnosis and during the first week than non-survivors, and that platelet cytochrome oxidase activity at moment of sepsis diagnosis and during the first week could be used as biomarker to predict the clinical outcome in septic patients

    The movement ecology of seagrasses

    Get PDF
    A movement ecology framework is applied to enhance our understanding of the causes, mechanisms and consequences of movement in seagrasses: marine, clonal, flowering plants. Four life-history stages of seagrasses can move: pollen, sexual propagules, vegetative fragments and the spread of individuals through clonal growth. Movement occurs on the water surface, in the water column, on or in the sediment, via animal vectors and through spreading clones. A capacity for long-distance dispersal and demographic connectivity over multiple timeframes is the novel feature of the movement ecology of seagrasses with significant evolutionary and ecological consequences. The space–time movement footprint of different life-history stages varies. For example, the distance moved by reproductive propagules and vegetative expansion via clonal growth is similar, but the timescales range exponentially, from hours to months or centuries to millennia, respectively. Consequently, environmental factors and key traits that interact to influence movement also operate on vastly different spatial and temporal scales. Six key future research areas have been identified

    Pharmacologic concentrations of linezolid modify oxidative phosphorylation function and adipocyte secretome

    Get PDF
    The oxidative phosphorylation system is important for adipocyte differentiation. Therefore, xenobiotics inhibitors of the oxidative phosphorylation system could affect adipocyte differentiation and adipokine secretion. As adipokines impact the overall health status, these xenobiotics may have wide effects on human health. Some of these xenobiotics are widely used therapeutic drugs, such as ribosomal antibiotics. Because of its similarity to the bacterial one, mitochondrial translation system is an off-target for these compounds. To study the influence of the ribosomal antibiotic linezolid on adipokine production, we analyzed its effects on adipocyte secretome. Linezolid, at therapeutic concentrations, modifies the levels of apolipoprotein E and several adipokines and proteins related with the extracellular matrix. This antibiotic also alters the global methylation status of human adipose tissue-derived stem cells and, therefore, its effects are not limited to the exposure period. Besides their consequences on other tissues, xenobiotics acting on the adipocyte oxidative phosphorylation system alter apolipoprotein E and adipokine production, secondarily contributing to their systemic effects

    La fauna del pleistoceno inferior de la sierra de Quibas (Abanilla, Murcia)

    Get PDF
    El yacimiento kárstico cuaternario de la Sierra de Quibas (Abanilla, Murcia) ha proporcionado una amplia lista faunística constituida por 53 especies repartidas entre gasterópodos, miriápodos, anfibios, reptiles, aves y mamíferos. En el conjunto de los gasterópodos destaca Palaeoglandina, un género que se extingue en el resto de Europa durante el Plioceno y que se mantiene como relicto en el Pleistoceno de la Península Ibérica. Dos de las especies de aves determinadas, Gypaetus barbatus y Gerontieus eremita, y una de las serpientes, Elaphe cf. E. sealaris, apenas se hallan representadas en el registro fósil. Hay además varias especies de aves cuya aparición en Quibas supone la primera cita en el Pleistoceno inferior de la Península Ibérica. Entre los mamíferos posee una especial relevancia la presencia del cercopitécido Macaca sylvanus. La asociación de los taxones Arvieola deuealíon, Castillomys rivas rivas, Elíomys intermedius, Equus altidens y Capra sp. aff. C. alba permite la correlación con Plines 1, Orce 3 y Venta Micena, entre otros. El yacimiento de Quibas puede situarse, por tanto, antes del final del Pleistoceno inferior, con una antigüedad entre 1.3 y 1 Ma. Por lo que se refiere a las condiciones paleoclimáticas, podemos inferir a partir de la asociación faunística un régimen xerófilo, muy semejante al actual en el área geográfica, aunque quizás con valores de humedad y temperatura algo superiores. El entorno de la cavidad kárstica estaba formado por roquedo calcáreo con áreas abiertas de matorral, pero en las proximidades se desarrollaron humedales y zonas arboladas, como así lo atestigua la presencia de aves y micromamíferos típicos de estos hábitats.The Quaternary karstic site of Sierra de Quibas (Abanilla, Murcia, Spain) has provided a wide faunallist with 53 species distributed among gastropods, myriapods, amphibians, reptiles, birds and mammals. Especially interesting among the gastropods is Palaeoglandina that became extinct in Europe during the Pliocene. It remains as a relict genus in the Pleistocene of the Iberian Peninsula. Two of the birds, Gypaetus barbatus and Geronticus eremita, and a snake, Elaphe cf. E. sealaris, are scarcely represented in the fossil record. For several birds, it is the first record in the Lower Pleistocene of the Iberian Peninsula. Among the mammals, the presence of the Cercopithecine Macaca sylvanus is especially relevant. The assemblage of the taxa Arvicola deucalion, Castillomys rivas rivas, Eliomys intermedius, Equus altidens and Capra sp. aff. C. alba allows the correlation with Plines 1, Orce 3 and Venta Micena, among other sites. Therefore Quibas can be clated before the end of Lower Pleistocene, between 1.3 and 1 Ma. A dry paleoclimatic regime, very similar to the current climate in the geographical area, though perhaps slightly wetter and warmer, can be inferred from the faunal assemblage. The environment of the karstic cavity was a rocky place with open brushwood areas, but in the proximities there were wetlands and woodlands, as can be inferred from the presence of birds and micromammals characteristic of these [email protected] [email protected]

    Mutations in the mitochondrial complex I assembly factor NDUFAF6 cause isolated bilateral striatal necrosis and progressive dystonia in childhood

    Get PDF
    Aim: To perform a deep phenotype characterisation in a pedigree of 3 siblings with Leigh syndrome and compound heterozygous NDUFAF6 mutations. Method: A multi-gene panel of childhood-onset basal ganglia neurodegeneration inherited conditions was analysed followed by functional studies in fibroblasts. Results: Three siblings developed gait dystonia in infancy followed by rapid progression to generalised dystonia and psychomotor regression. Brain magnetic resonance showed symmetric and bilateral cytotoxic lesions in the putamen and proliferation of the lenticular-striate arteries, latter spreading to the caudate and progressing to cavitation and volume loss. We identified a frameshift novel change (c.554_558delTTCTT; p.Tyr187AsnfsTer65) and a pathogenic missense change (c.371T>C; p.Ile124Thr) in the NDUFAF6 gene, which segregated with an autosomal recessive inheritance within the family. Patient mutations were associated with the absence of the NDUFAF6 protein and reduced activity and assembly of mature complex I in fibroblasts. By functional complementation assay, the mutant phenotype was rescued by the canonical version of the NDUFAF6. A literature review of 14 NDUFAF6 patients showed a consistent phenotype of an early childhood insidious onset neurological regression with prominent dystonia associated with basal ganglia degeneration and long survival. Interpretation: NDUFAF6-related Leigh syndrome is a relevant cause of childhood onset dystonia and isolated bilateral striatal necrosis. By genetic complementation, we could demonstrate the pathogenicity of novel genetic variants in NDUFAF6
    corecore