2,133 research outputs found
Arsenic uptake by gypsum and calcite: Modeling and probing by neutron and x-ray scattering
Here we report on two structural studies performed on As-doped gypsum (CaSO4
2H2O) and calcite (CaCO3), using neutron (D20-ILL) and x-ray (ID11-ESRF)
diffraction data and EXAFS (BM8-ESRF). The aim of this study is to determine
whether As gets into the bulk of gypsum and calcite structures or is simply
adsorbed on the surface. Different mechanisms of substitution are used as
hypotheses. The combined Rietveld analysis of neutron and x-ray diffraction
data shows an expansion of the unit cell volume proportional to the As
concentration within the samples. DFT-based simulations confirm the increase of
the unit cell volume proportional to the amount of carbonate or sulphate groups
substituted. Interpolation of the experimental Rietveld data allows us to
distinguish As substituted within the structure from that adsorbed on the
surface of both minerals.Comment: Proceedings of the Eighth International Conference on Neutron
Scatterin
Improvement in Vision: A New Goal for Treatment of Hereditary Retinal Degenerations
Introduction: Inherited retinal degenerations (IRDs) have long been considered untreatable and incurable. Recently, one form of early-onset autosomal recessive IRD, Leber congenital amaurosis (LCA) caused by mutations in RPE65 (retinal pigment epithelium-specific protein 65 kDa) gene, has responded with some improvement of vision to gene augmentation therapy and oral retinoid administration. This early success now requires refinement of such therapeutics to fully realize the impact of these major scientific and clinical advances. Areas covered: Progress toward human therapy for RPE65-LCA is detailed from the understanding of molecular mechanisms to preclinical proof-of-concept research to clinical trials. Unexpected positive and complicating results in the patients receiving treatment are explained. Logical next steps to advance the clinical value of the therapeutics are suggested. Expert opinion: The first molecularly based early-phase therapies for an IRD are remarkably successful in that vision has improved and adverse events are mainly associated with surgical delivery to the subretinal space. Yet, there are features of the gene augmentation therapeutic response, such as slowed kinetics of night vision, lack of foveal cone function improvement and relentlessly progressive retinal degeneration despite therapy, that still require research attention
Lightning Impulse Current Tests on Conductive Fabrics
Submitted to "Journal of Industrial Textiles"The large amount of electric current associated to lightning discharges is hazardous for living beings, equipment, structures and buildings. To protect those targets against lightning are used Lightning Protection Systems (LPS). However, there are some temporary outdoor activities and backcountry places where an adequate LPS cannot be set up mainly due to the large dimensions of its components and its heavy weight. On the search of light weight lightning protection materials that can be used as part of special LPS, we research some types of electroconductive fabrics by applying high lightning impulse currents in laboratory. The fabric samples checked were pieces of 10 cm x 10 cm: two rip-stop type, a plain-weave, a non-woven and a carbon-impregnated polymeric, all of them obtained commercially. Under laboratory conditions, these samples were subject to subsequent lightning impulse currents registering the voltage and current signals. Optical and scanning electron microscope inspections were performed after tests. Despite some changes visualized as marks left on the fabric surface, the results show that investigated conductive textiles can endure ground currents produced by atmospheric lightning since they withstand the several applied laboratory lightning impulse currents. The outcomes suggest that the weave pattern of the conductive fabric influences the lightning current tolerance, enabling some conductive fabrics to be used in heavy-current applications and as part of personal LPS for outdoor, backcountry and mobile shelters, particularly when lightweight and portability are mandatory
Gene flow in the Antarctic bivalve Aequiyoldia eightsii (Jay, 1839) suggests a role for the Antarctic Peninsula Coastal Current in larval dispersal
The Antarctic Circumpolar Current (ACC) dominates the open-ocean circulation of the Southern Ocean, and both isolates and connects the Southern Ocean biodiversity. However, the impact on biological processes of other Southern Ocean currents is less clear. Adjacent to the West Antarctic Peninsula (WAP), the ACC flows offshore in a northeastward direction, whereas the Antarctic Peninsula Coastal Current (APCC) follows a complex circulation pattern along the coast, with topographically influenced deflections depending on the area. Using genomic data, we estimated genetic structure and migration rates between populations of the benthic bivalve Aequiyoldia eightsii from the shallows of southern South America and the WAP to test the role of the ACC and the APCC in its dispersal. We found strong genetic structure across the ACC (between southern South America and Antarctica) and moderate structure between populations of the WAP. Migration rates along the WAP were consistent with the APCC being important for species dispersal. Along with supporting current knowledge about ocean circulation models at the WAP, migration from the tip of the Antarctic Peninsula to the Bellingshausen Sea highlights the complexities of Southern Ocean circulation. This study provides novel biological evidence of a role of the APCC as a driver of species dispersal and highlights the power of genomic data for aiding in the understanding of the influence of complex oceanographic processes in shaping the population structure of marine species
The Holocene Cedrus pollen record from Sierra Nevada (S Spain), a proxy for climate change in N Africa
Comprehending the effects of climate variability and disturbance on forested ecosystems is paramount to successfully managing forest environments under future climate scenarios (e.g., global warming, aridi-fication increase). Changes in fossil pollen abundance in sedimentary archives record past vegetation dynamics at regional scales, mainly related to climate changes and, in the last few millennia, to human impact. Pollen records can thus provide long databases with information on how the environment reacted to climate change before the historical record. In this study, we synthesized fossil pollen data from seven sites from the Sierra Nevada in southern Spain to investigate the response of forests in the western Mediterranean area to millennial-scale climate changes and to human impact during the Holocene. In particular, here we focused on Cedrus pollen abundances, which most-likely originated from Northern Africa and were carried to Sierra Nevada by wind. Cedrus pollen has received little attention in the Iberian Peninsula palynological records, for it occurs in low concentrations and has an African source, and thus this article explores the potential to reconstruct its past history and climate. Although Cedrus abundances are generally lower than 1% in the studied pollen samples, a comparison with North African (Moroccan) Cedrus pollen records shows similar trends at long- and short-term time-scales. Therefore, this record could be used as a proxy for changes in this forest species in North Africa. As observed in the Sierra Nevada synthetic record, the increasing trend of Cedrus pollen during the Middle and Late Holocene closely correlates with decreasing summer insolation. This would have produced overall cooler annual temperatures in Northern Africa (Middle Atlas and Rif Mountains) as well as lower summer evaporation, benefiting the growth of this cool-adapted montane tree species while increasing available moisture during the summer, which is critical for this water-demanding species. Millennial-scale variability also characterizes the Sierra Nevada Cedrus synthetic pollen record. Cedrus abundance oscillations co-vary with well-known millennial-scale climatic variability that controlled cedar abundance and altitudinal distribution in montane areas of N Africa. (C) 2020 Elsevier Ltd. All rights reserved.Peer reviewe
Climatic control on the Holocene hydrology of a playa-lake system in the western Mediterranean
Evaporitic lakes such as playa-lakes are characteristic of many arid regions and are unique environments with respect to fauna and flora, while being very vulnerable to climate and environmental fluctuations and threatened by the current global change scenario. Water balance oscillations in these systems can trigger the precipitation or dissolution of different evaporitic minerals, negatively impacting local biodiversity and economic activities. Here, we study the sedimentary record of a small saline pond from a playa-lake complex in southwestern Iberia in order to reconstruct the paleohydrological evolution of this area and assess potential anthropogenic disturbances. The different proxies studied in the-11.9 ky old sedimentary record of the Laguna de la Ballestera suggest that the greatest lake extension and the highest water levels occurred during the Early Holocene, pointing to the wettest period of the record. Climate transitioned towards more arid conditions during the Middle Holocene, and even more dramatically during the Late Holocene. In this last stage the wetland surface and the water level largely diminished and gypsum precipitation gradually increased pointing towards a negative precipitation/ evapotranspiration balance and lowest water levels. Summer desiccation likely occurred under this scenario, especially after-1.0-0.9 cal ky BP coeval with the Medieval Climate Anomaly, when gypsum content started to rise abruptly. However, this significant gypsum precipitation was only associated with a massive drop in the siliciclastic content and scarce carbonates (dolomite and calcite) during the last-400 years. This evidence suggests a shift from a (semi) permanent to a temporal/seasonal hydrological regime. The environmental evolution of this wetland responded to the general climatic evolution of the western Mediterranean during the Holocene, being mostly controlled by changes in insolation. Our data also show that the environmental response of the studied wetland to natural climate variations was only significantly disturbed by human activities since the 20th century, especially in the second half of the century, deduced by abrupt fluctuations in the siliciclastic, gypsum and organic content in the sediments, as well as by the enhanced sedimentary accumulation rates, probably as a response to changes in the hydroperiod of the lake and in the catchment land use.Peer reviewe
Paleohydrological dynamics in the Western Mediterranean during the last glacial cycle
The transitional regions between the low and high latitudes of the Northern Hemisphere are highly vulnerable to future climate change yet most of the current climate models usually diverge in their projections. To better understand the dynamics in these regions, the reconstruction of past hydrological fluctuations and precipitation patterns is of paramount importance to accurately constrain present and future climate scenarios. In this study, we investigated paleohydrological dynamics in the western Mediterranean region, a transitional zone between low-mid latitudes and Atlantic - Mediterranean realms. We reconstruct precipitation and moisture source changes during the last -35 ka in order to propose the potential mechanisms driving these oscillations. To do so, we use hydrogen isotopes from sedimentary leaf waxes, more specifically the C31 n-alkane homologue, and a precipitation proxy based on previously published pollen data from a sedimentary core (Padul-15-05) in southern Iberia (Padul wetland -37-N). With this combination we disentangle the coupled effect of precipitation amount and source on the hydrogen isotopic signature of the studied C31 n-alkane record. Our results show three main periods characterized by different precipitation patterns. Low precipitation, mainly linked to a significant contribution from an isotopically-enriched Mediterranean precipitation source, occurred from -30 to -15.5 ka BP and during the last -5 ka, whereas enhanced precipitation with a predominant isotopically-depleted Atlantic precipitation source prevailed from -15.5 to -5 ka BP. This latter stage is here defined as the Western Mediterranean Humid Period (WMHP). In addition, some occasional millennial-scale opposite precipitation patterns can be observed during these climatically distinct periods. These changes in the source of precipitation were likely coupled to a shift in the main rainy season from winter, when Atlantic precipitation prevailed, to late winter-early spring, when the contribution of Mediterranean moisture is higher. Comparison between the studied mid-latitude terrestrial Padul-15-05 core and a low-latitude marine record offshore of northwestern Africa shows clear long-term synchronous responses of both western Mediterranean precipitation and western African monsoon systems to northern Hemisphere atmospheric dynamics, ultimately controlled by orbital forcing and ice-sheets fluctuations.Peer reviewe
Human Retinal Gene Therapy for Leber Congential Amaurosis Shows Advancing Retinal Degeneration Despite Enduring Visual Improvement
Leber congenital amaurosis (LCA) associated with retinal pigment epithelium-specific protein 65 kDa (RPE65) mutations is a severe hereditary blindness resulting from both dysfunction and degeneration of photoreceptors. Clinical trials with gene augmentation therapy have shown partial reversal of the dysfunction, but the effects on the degeneration are not known. We evaluated the consequences of gene therapy on retinal degeneration in patients with RPE65-LCA and its canine model. In untreated RPE65-LCA patients, there was dysfunction and degeneration of photoreceptors, even at the earliest ages. Examined serially over years, the outer photoreceptor nuclear layer showed progressive thinning. Treated RPE65-LCA showed substantial visual improvement in the short term and no detectable decline from this new level over the long term. However, retinal degeneration continued to progress unabated. In RPE65-mutant dogs, the first one-quarter of their lifespan showed only dysfunction, and there was normal outer photoreceptor nuclear layer thickness retina-wide. Dogs treated during the earlier dysfunction-only stage showed improved visual function and dramatic protection of treated photoreceptors from degeneration when measured 5–11 y later. Dogs treated later during the combined dysfunction and degeneration stage also showed visual function improvement, but photoreceptor loss continued unabated, the same as in human RPE65-LCA. The results suggest that, in RPE65 disease treatment, protection from visual function deterioration cannot be assumed to imply protection from degeneration. The effects of gene augmentation therapy are complex and suggest a need for a combinatorial strategy in RPE65-LCA to not only improve function in the short term but also slow retinal degeneration in the long term
Treatment Strategy with Gene Editing for Late-onset Retinal Degeneration Caused by a Founder Variant in C1QTNF5
AbstractPurpose: Genome editing is an emerging group of technologies with the potential to ameliorate dominant, monogenic human diseases such as late-onset retinal degeneration (L-ORD). The goal of this study was to identify disease stages and retinal locations optimal for evaluating the efficacy of a future genome editing trial.Methods: Twenty five L-ORD patients (age range, 33-77 years; median age, 59 years) harboring the founder variant S163R in C1QTNF5 were enrolled from three centers in the United Kingdom and United States. Patients were examined with widefield optical coherence tomography (OCT) and chromatic perimetry under dark-adapted and light-adapted conditions to derive phenomaps of retinal disease. Results were analyzed with a model of a shared natural history of a single delayed exponential across all subjects and all retinal locations.Results: Critical age for the initiation of photoreceptor loss ranged from 48 years at the temporal paramacular retina to 74 years at the inferior midperipheral retina. Subretinal deposits (sRET-Ds) became more prevalent as critical age was approached. Subretinal pigment epithelial deposits (sRPE-Ds) were detectable in the youngest patients showing no other structural or functional abnormalities at the retina. The sRPE-D thickness continuously increased, reaching 25 µm in the extrafoveal retina and 19 µm in the fovea at critical age. Loss of light sensitivity preceded shortening of outer segments and loss of photoreceptors by more than a decade.Conclusions: Retinal regions providing an ideal treatment window exist across all severity stages of L-ORD
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