Neuronal Intermediate Filaments in Amyotrophic Lateral Sclerosis

Neuronal intermediate filaments (NIFs) are the most abundant cytoskeletal element in mature neurons. They are composed of different protein subunits encoded by separate genes such as neurofilament light chain (NFL), neurofilament medium chain (NFM), neurofilament heavy chain (NFH), ɑ‐internexin and peripherin. NIFs are dynamic structures playing important functions in cell architecture and differentiation, interactions between proteins or subcellular organelles, and in axonal calibre determination and myelination. Consequently, their presence modulates electrophysiological properties of axons. NIFs have long been assigned a role in the pathogenesis of amyotrophic lateral sclerosis (ALS). Indeed, accumulation and abnormal phosphorylation of NIF subunits in motor neuron are one of the major pathological features in both sporadic and familial forms of the disease. Moreover, mutations in the NFH and peripherin genes and elevated cerebrospinal fluid NIF levels reported in ALS cases, associated with studies in transgenic mice, provided the evidence that primary defects in NIFs could be causative for motor neuron disease. However, the processes leading to the NIF abnormalities and the links to the pathogenesis of ALS remain unclear, leaving a challenging open field for further investigations in this highly disabilitating disease. Here, we review the main characteristics of these NIFs and their involvement in the pathomechanisms of ALS

Chewing, dental morphology and wear in tapirs (Tapirus spp.) and a comparison of free-ranging and captive specimens

Feeding practice in herbivorous mammals can impact their dental wear, due to excessive or irregular abrasion. Previous studies indicated that browsing species display more wear when kept in zoos compared to natural habitats. Comparable analyses in tapirs do not exist, as their dental anatomy and chewing kinematics are assumed to prevent the use of macroscopic wear proxies such as mesowear. We aimed at describing tapir chewing, dental anatomy and wear, to develop a system allowing comparison of free-ranging and captive specimens even in the absence of known age. Video analyses suggest that in contrast to other perissodactyls, tapirs have an orthal (and no lateral) chewing movement. Analysing cheek teeth from 74 museum specimens, we quantified dental anatomy, determined the sequence of dental wear along the tooth row, and established several morphometric measures of wear. In doing so, we showcase that tapir maxillary teeth distinctively change their morphology during wear, developing a height differential between less worn buccal and more worn lingual cusps, and that quantitative wear corresponds to the eruption sequence. We demonstrate that mesowear scoring shows a stable signal during initial wear stages but results in a rather high mesowear score compared to other browsing herbivores. Zoo specimens had lesser or equal mesowear scores as specimens from the wild; additionally, for the same level of third molar wear, premolars and other molars of zoo specimens showed similar or less wear compared specimens from the wild. While this might be due to the traditional use of non-roughage diet items in zoo tapirs, these results indicate that in contrast to the situation in other browsers, excessive tooth wear appears to be no relevant concern in ex situ tapir management

Exploring the faint young Sun problem and the possible climates of the Archean Earth with a 3-D GCM

Different solutions have been proposed to solve the “faint young Sun problem,” defined by the fact that the Earth was not fully frozen during the Archean despite the fainter Sun. Most previous studies were performed with simple 1-D radiative convective models and did not account well for the clouds and ice-albedo feedback or the atmospheric and oceanic transport of energy. We apply a global climate model (GCM) to test the different solutions to the faint young Sun problem. We explore the effect of greenhouse gases (CO2 and CH4), atmospheric pressure, cloud droplet size, land distribution, and Earth's rotation rate. We show that neglecting organic haze, 100 mbar of CO2 with 2 mbar of CH4 at 3.8 Ga and 10 mbar of CO2 with 2 mbar of CH4 at 2.5 Ga allow a temperate climate (mean surface temperature between 10°C and 20°C). Such amounts of greenhouse gases remain consistent with the geological data. Removing continents produces a warming lower than +4°C. The effect of rotation rate is even more limited. Larger droplets (radii of 17 μm versus 12 μm) and a doubling of the atmospheric pressure produce a similar warming of around +7°C. In our model, ice-free water belts can be maintained up to 25°N/S with less than 1 mbar of CO2 and no methane. An interesting cloud feedback appears above cold oceans, stopping the glaciation. Such a resistance against full glaciation tends to strongly mitigate the faint young Sun problem.Engineering and Applied Science

Dental microwear texture analysis correlations in guinea pigs (Cavia porcellus) and sheep (Ovis aries) suggest that dental microwear texture signal consistency is species-specific

Dental microwear texture (DMT) analysis is used to differentiate abrasive dental wear patterns in many species fed different diets. Because DMT parameters all describe the same surface, they are expected to correlate with each other distinctively. Here, we explore the data range of, and correlations between, DMT parameters to increase the understanding of how this group of proxies records wear within and across species. The analysis was based on subsets of previously published DMT analyses in guinea pigs, sheep, and rabbits fed either a natural whole plant diet (lucerne, grass, bamboo) or pelleted diets with or without added quartz abrasives (guinea pigs and rabbits: up to 45 days, sheep: 17 months). The normalized DMT parameter range (P4: 0.69   0.25; M2: 0.83   0.16) and correlation coefficients (P4: 0.50   0.31; M2: 0.63   0.31) increased along the tooth row in guinea pigs, suggesting that strong correlations may be partially explained by data range. A comparison between sheep and guinea pigs revealed a higher DMT data range in sheep (0.93   0.16; guinea pigs: 0.47   0.29), but this did not translate into more substantial correlation coefficients (sheep: 0.35   0.28; guinea pigs: 0.55   0.32). Adding rabbits to an interspecies comparison of low abrasive dental wear (pelleted lucerne diet), the softer enamel of the hypselodont species showed a smaller data range for DMT parameters (guinea pigs 0.49   0.32, rabbit 0.19   0.18, sheep 0.78   0.22) but again slightly higher correlations coefficients compared to the hypsodont teeth (guinea pigs 0.55   0.31, rabbits 0.56   0.30, sheep 0.42   0.27). The findings suggest that the softer enamel of fast-replaced ever-growing hypselodont cheek teeth shows a greater inherent wear trace consistency, whereas the harder enamel of permanent and non-replaced enamel of hypsodont ruminant teeth records less coherent wear patterns. Because consistent diets were used across taxa, this effect cannot be ascribed to the random overwriting of individual wear traces on the more durable hypsodont teeth. This matches literature reports on reduced DMT pattern consistency on harder materials; possibly, individual wear events become more random in nature on harder material. Given the species-specific differences in enamel characteristics, the findings suggest a certain species-specificity of DMT patterns

Body condition and ruminal morphology responses of free-ranging impala ( Aepyceros melampus ) to changes in diet

Variation of the intraruminal papillation pattern with diet quality has been described in many ruminant species, but the use of papillation measures as a proxy for habitat quality and nutritional status of animals has not been evaluated. We compared various measures of body condition (body mass, body condition score, kidney fat index, bone marrow fat index, adrenal mass, kidney to adrenal mass ratio), diet quality (%browse, protein and fibre content) and rumen papillation in 106 impalas (Aepyceros melampus) from four different locations in Zimbabwe. The various condition proxies indicated that periods of low diet quality are characterised by a high proportion of browse in the diet of this species. Animals with a high proportion of browse had more voluminous rumens, suggesting a compensation for lower diet quality by increased intake. Macroscopic papillation indices did not yield meaningful significant correlations with diet quality or body condition proxies, and hence, their use for estimating habitat or body condition cannot be advocated. In contrast to previous histological reports, ballooning cells of the Stratum corneum of the ruminal mucosa were more prominent in animals on lower-quality diets. There were significant correlations of the kidney to adrenal mass ratio with other body conditions and with diet quality indices, suggesting that poor body condition and low diet quality represent stressful situations

Abundant electrical myotonia and left ventricular noncompaction: Unusual features of Danon disease due to a novel mutation in LAMP2 gene

International audienc

The PreVOCA experiment: modeling the lower troposphere in the Southeast Pacific

The Preliminary VOCALS Model Assessment (PreVOCA) aims to assess contemporary atmospheric modeling of the subtropical South East Pacific, with a particular focus on the clouds and the marine boundary layer (MBL). Models results from fourteen modeling centers were collected including operational forecast models, regional models, and global climate models for the month of October 2006. Forecast models and global climate models produced daily forecasts, while most regional models were run continuously during the study period, initialized and forced at the boundaries with global model analyses. Results are compared in the region from 40° S to the equator and from 110° W to 70° W, corresponding to the Pacific coast of South America. Mean-monthly model surface winds agree well with QuikSCAT observed winds and models agree fairly well on mean weak large-scale subsidence in the region next to the coast. However they have greatly differing geographic patterns of mean cloud fraction with only a few models agreeing well with MODIS observations. Most models also underestimate the MBL depth by several hundred meters in the eastern part of the study region. The diurnal cycle of liquid water path is underestimated by most models at the 85° W 20° S stratus buoy site compared with satellite, consistent with previous modeling studies. The low cloud fraction is also underestimated during all parts of the diurnal cycle compared to surface-based climatologies. Most models qualitatively capture the MBL deepening around 15 October 2006 at the stratus buoy, associated with colder air at 700 hPa

The partitioning of poleward energy transport response between the atmosphere and Ekman flux to prescribed surface forcing in a simplified GCM

Recent studies have indicated that ocean circulation damps the atmospheric energy transport response to hemispherically differential energy perturbations, thereby muting the shifts of the Inter-Tropical Convergence Zone (ITCZ). Here, we focus on the potential role of Ekman heat transport in modulating this atmospheric response. An idealized representation of Ekman-driven heat transport (FE) is included in an aquaplanet slab ocean coupled to a gray radiation atmospheric model. We first alter the strength of FE in the control climate by tuning the gross stability of the Ekman layer SE. For a wide range of FE, the total poleward transport of energy remains nearly unchanged, but the ocean transports an increasing share for larger SE. The control climate is then perturbed by adding surface cooling in the Southern Hemisphere and warming in the Northern Hemisphere. The Ekman coupling damps the atmospheric energy transport response, as in previous coupled model experiments with full ocean dynamics. The ratio of the changes in Ekman to atmospheric energy transport is determined by the ratio of the gross stability in the Ekman layer to the atmosphere in the control climate, and is insensitive to the amplitude and location of forcing. We find that an unrealistically large SE is needed to reproduce the ratio of the changes in cross-equatorial oceanic to atmospheric energy transport in fully coupled models. The limited damping effect of Ekman transport highlights the need to examine the roles of deep circulation and subtropical gyres, as well as ocean heat uptake processes

The extreme physical properties of the CoRoT-7b super-Earth

International audience► Here, we discuss the extreme physical properties possible for the first characterized rocky super-Earth, CoRoT-7b ( = 1.58 , = 5.7 ). ► We make the working hypothesis that the planet is rocky with no volatiles in its atmosphere, and derive the physical properties that result. ► The dayside is very hot (2500 K at the sub-stellar point) while the nightside is very cold (∼ 50 K). The sub-stellar point is as hot as the tungsten filament of an incandescent bulb, resulting in the melting and distillation of silicate rocks and the formation of a lava ocean. ► These possible features of CoRoT-7b should be common to many small and hot planets, including Kepler-10b. They define a new class of objects that we propose to name ''Lava-ocean planets''

Carbon stable isotope analysis of cereal remains as a way to reconstruct water availability: preliminary results

Reconstructing past water availability, both as rainfall and irrigation, is important to answer questions about the way society reacts to climate and its changes and the role of irrigation in the development of social complexity. Carbon stable isotope analysis of archaeobotanical remains is a potentially valuable method for reconstructing water availability. To further define the relationship between water availability and plant carbon isotope composition and to set up baseline values for the Southern Levant, grains of experimentally grown barley and sorghum were studied. The cereal crops were grown at three stations under five different irrigation regimes in Jordan. Results indicate that a positive but weak relationship exists between irrigation regime and total water input of barley grains, but no relationship was found for sorghum. The relationship for barley is site-specific and inter-annual variation was present at Deir ‘Alla, but not at Ramtha and Khirbet as-Samra