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Sentinel-2 satellite data enables multispectral monitoring of the earth at a high temporal revisit rate. Combining this information with a network of optical ground measurements enables a more detailed and a more complete understanding of terrestrial ecosystems. However, independent optical ground measurements often lack consistency, especially when comparing different sites in geographically remote locations. Using the very high temporal and spectral resolution offered by the automated field spectrometer systems FloX and RoX (Fluorescence Box and Reflectance Box, respectively, JB-Hyperspectral Devices GmbH, Duesseldorf, Germany), we investigated continuous time series ranging over three years and in ten different locations across Europe, Africa, America and Asia. The continuous records of ground-measured reflectance were first validated against Sentinel-2 top of canopy (TOC) reflectance to evaluate the consistency of the in-situ network. Our results suggest a good agreement of ground-measured reflectance with Sentinel-2 TOC reflectance in vegetation and snow with R2 around 0.79 in the 833 nm band and R2 up to 0.94 in the bands around 559 nm and 492 nm, demonstrating good consistency across the network. Spatial misalignment of Sentinel-2 pixel-sizes with respect to the different footprint sizes of the ten automated spectrometers on the ground, atmospheric uncertainties, sub-optimal instrument setup and spatial-temporal variable landscape heterogeneity were identified as the most relevant sources of uncertainties in the network. Comparing the Normalized Difference Vegetation Index (NDVI), Transformed Chlorophyll Absorption in Reflectance Index (TCARI) and Enhanced Vegetation Index (EVI) between ground and satellite revealed a decreasing agreement with increasing complexity of index formulation. The best agreement between satellite and ground was exhibited by NDVI with R2 around 0.96 and relative error of 4.3% investigating vegetation and snow across all ten sites. Fu

Mucopolysaccharidosis type I Hurler (MPSIH) is characterized by severe and progressive skeletal dysplasia that is not fully addressed by allogeneic hematopoietic stem cell transplantation (HSCT). Autologous hematopoietic stem progenitor cell-gene therapy (HSPC-GT) provides superior metabolic correction in patients with MPSIH compared with HSCT; however, its ability to affect skeletal manifestations is unknown. Eight patients with MPSIH (mean age at treatment: 1.9 years) received lentiviral-based HSPC-GT in a phase 1/2 clinical trial (NCT03488394). Clinical (growth, measures of kyphosis and genu velgum), functional (motor function, joint range of motion), and radiological [acetabular index (AI), migration percentage (MP) in hip x-rays and MRIs and spine MRI score] parameters of skeletal dysplasia were evaluated at baseline and multiple time points up to 4 years after treatment. Specific skeletal measures were retrospectively compared with an external cohort of HSCT-treated patients. At a median follow-up of 3.78 years after HSPC-GT, all patients treated with HSPC-GT exhibited longitudinal growth within WHO reference ranges and a median height gain greater than that observed in patients treated with HSCT after 3-year follow-up. Patients receiving HSPC-GT experienced complete and earlier normalization of joint mobility compared with patients treated with HSCT. Mean AI and MP showed progressive decreases after HSPC-GT, suggesting a reduction in acetabular dysplasia. Typical spine alterations measured through a spine MRI score stabilized after HSPC-GT. Clinical, functional, and radiological measures suggested an early beneficial effect of HSPC-GT on MPSIH-typical skeletal features. Longer follow-up is needed to draw definitive conclusions on HSPC-GT's impact on MPSIH skeletal dysplasia.

Climate extremes are on the rise. Impacts of extreme climate and weather events on ecosystem services and ultimately human well-being can be partially attenuated by the organismic, structural, and functional diversity of the affected land surface. However, the ongoing transformation of terrestrial ecosystems through intensified exploitation and management may put this buffering capacity at risk. Here, we summarise the evidence that reductions in biodiversity can destabilise the functioning of ecosystems facing climate extremes. We then explore if impaired ecosystem functioning could, in turn, exacerbate climate extremes. We argue that only a comprehensive approach, incorporating both ecological and hydrometeorological perspectives, enables to understand and predict the entire feedback system between altered biodiversity and climate extremes. This ambition, however, requires a reformulation of current research priorities to emphasise the bidirectional effects that link ecology and atmospheric processes.

Sun-induced chlorophyll fluorescence (SIF) is a promising new tool for remotely estimating photosynthesis. However, the degree to which incoming solar radiation and the structure of the canopy rather than leaf physiology contribute to SIF variations is still not well characterized. Therefore, we investigated relationships between SIF and variables that at least partly capture the canopy structure component of SIF. For this, we relied on high-quality SIF observations from ground-based instruments, high-resolution airborne SIF imagery and the most recent satellite SIF products to cover large ranges in spatial and temporal resolution and diverse ecosystems. We found that the canopy structure-related near-infrared reflectance of vegetation multiplied by incoming sunlight (NIRVP) is a robust proxy for far-red SIF across a wide range of spatial and temporal scales. Our findings indicate that contributions from leaf physiology to SIF variability are small compared to the structure and radiation components. Also, NIRVP captured spatio-temporal patterns of canopy photosynthesis better than SIF, which seems to be mostly due to the greater retrieval noise of SIF. Compared to other relevant structural SIF proxies, NIRVP showed more robust relationships to SIF, especially at the global scale. Our results highlight the promise of using widely available NIRVP data for vegetation monitoring and also indicate the potential of using SIF and NIRVP in combination to extract physiological information from SIF.

Sun-induced fluorescence in the far-red region (SIF) is increasingly used as a remote and proximal-sensing tool capable of tracking vegetation gross primary production (GPP). However, the use of SIF to probe changes in GPP is challenged during extreme climatic events, such as heatwaves. Here, we examined how the 2018 European heatwave (HW) affected the GPP–SIF relationship in evergreen broadleaved trees with a relatively invariant canopy structure. To do so, we combined canopy-scale SIF measurements, GPP estimated from an eddy covariance tower, and active pulse amplitude modulation fluorescence. The HW caused an inversion of the photosynthesis–fluorescence relationship at both the canopy and leaf scales. The highly nonlinear relationship was strongly shaped by nonphotochemical quenching (NPQ), that is, a dissipation mechanism to protect from the adverse effects of high light intensity. During the extreme heat stress, plants experienced a saturation of NPQ, causing a change in the allocation of energy dissipation pathways towards SIF. Our results show the complex modulation of the NPQ–SIF–GPP relationship at an extreme level of heat stress, which is not completely represented in state-of-the-art coupled radiative transfer and photosynthesis models.

While the eddy covariance (EC) technique is a well-established method for measuring water fluxes (i.e., evaporation or 'evapotranspiration’, ET), the measurement is susceptible to many uncertainties. One such issue is the potential underestimation of ET when relative humidity (RH) is high (>70%), due to low-pass filtering with some EC systems. Yet, this underestimation for different types of EC systems (e.g. open-path or closed-path sensors) has not been characterized for synthesis datasets such as the widely used FLUXNET2015 dataset. Here, we assess the RH-associated underestimation of latent heat fluxes (LE, or ET) from different EC systems for 163 sites in the FLUXNET2015 dataset. We found that the LE underestimation is most apparent during hours when RH is higher than 70%, predominantly observed at sites using closed-path EC systems, but the extent of the LE underestimation is highly site-specific. We then propose a machine learning based method to correct for this underestimation, and compare it to two energy balance closure based LE correction approaches (Bowen ratio correction, BRC, and attributing all errors to LE). Our correction increases LE by 189% for closed-path sites at high RH (>90%), while BRC increases LE by around 30% for all RH conditions. Additionally, we assess the influence of these corrections on ET-based transpiration (T) estimates using two different ET partitioning methods. Results show opposite responses (increasing vs. slightly decreasing T-to-ET ratios, T/ET) between the two methods when comparing T based on corrected and uncorrected LE. Overall, our results demonstrate the existence of a high RH bias in water fluxes in the FLUXNET2015 dataset and suggest that this bias is a pronounced source of uncertainty in ET measurements to be considered when estimating ecosystem T/ET and WUE.

Both carbon dioxide uptake and albedo of the land surface affect global climate. However, climate change mitigation by increasing carbon uptake can cause a warming trade-off by decreasing albedo, with most research focusing on afforestation and its interaction with snow. Here, we present carbon uptake and albedo observations from 176 globally distributed flux stations. We demonstrate a gradual decline in maximum achievable annual albedo as carbon uptake increases, even within subgroups of non-forest and snow-free ecosystems. Based on a paired-site permutation approach, we quantify the likely impact of land use on carbon uptake and albedo. Shifting to the maximum attainable carbon uptake at each site would likely cause moderate net global warming for the first approximately 20 years, followed by a strong cooling effect. A balanced policy co-optimizing carbon uptake and albedo is possible that avoids warming on any timescale, but results in a weaker long-term cooling effect.

Portfolio diversification (PD) is attracting increasing attention, as it is becoming more difficult to optimize portfolios due to growing financial markets integration. To highlight the evolution of PD literature, we perform a meta-literature review on 242 articles published between 1974 and 2022. We identify the most influential aspects of the literature and perform co-authorship, cartographic and co-citation analyses. We identify four main research clusters, as well as the most influential articles in the field. We map the different streams of the literature, visualizing different focus areas and trends, and identify more than 60 unanswered research questions. Findings will facilitate scholars focusing on under-investigated areas.

Both carbon dioxide uptake and albedo of the land surface affect global climate. However, climate change mitigation by increasing carbon uptake can cause a warming trade-off by decreasing albedo, with most research focusing on afforestation and its interaction with snow. Here, we present carbon uptake and albedo observations from 176 globally distributed flux stations. We demonstrate a gradual decline in maximum achievable annual albedo as carbon uptake increases, even within subgroups of non-forest and snow-free ecosystems. Based on a paired-site permutation approach, we quantify the likely impact of land use on carbon uptake and albedo. Shifting to the maximum attainable carbon uptake at each site would likely cause moderate net global warming for the first approximately 20 years, followed by a strong cooling effect. A balanced policy co-optimizing carbon uptake and albedo is possible that avoids warming on any timescale, but results in a weaker long-term cooling effect.

Plant transpiration links physiological responses of vegetation to water supply and demand with hydrological, energy, and carbon budgets at the land-atmosphere interface. However, despite being the main land evaporative flux at the global scale, transpiration and its response to environmental drivers are currently not well constrained by observations. Here we introduce the first global compilation of whole-plant transpiration data from sap flow measurements (SAPFLUXNET, https://sapfluxnet.creaf.cat/, last access: 8 June 2021). We harmonized and quality-controlled individual datasets supplied by contributors worldwide in a semi-automatic data workflow implemented in the R programming language. Datasets include sub-daily time series of sap flow and hydrometeorological drivers for one or more growing seasons, as well as metadata on the stand characteristics, plant attributes, and technical details of the measurements. SAPFLUXNET contains 202 globally distributed datasets with sap flow time series for 2714 plants, mostly trees, of 174 species. SAPFLUXNET has a broad bioclimatic coverage, with woodland/shrubland and temperate forest biomes especially well represented (80% of the datasets). The measurements cover a wide variety of stand structural characteristics and plant sizes. The datasets encompass the period between 1995 and 2018, with 50% of the datasets being at least 3 years long. Accompanying radiation and vapour pressure deficit data are available for most of the datasets, while on-site soil water content is available for 56% of the datasets. Many datasets contain data for species that make up 90% or more of the total stand basal area, allowing the estimation of stand transpiration in diverse ecological settings. SAPFLUXNET adds to existing plant trait datasets, ecosystem flux networks, and remote sensing products to help increase our understanding of plant water use, plant responses to drought, and ecohydrological processes. SAPFLUXNET version 0.1.5 is freely a

The leaf economics spectrum and the global spectrum of plant forms and functions3 revealed fundamental axes of variation in plant traits, which represent different ecological strategies that are shaped by the evolutionary development of plant species. Ecosystem functions depend on environmental conditions and the traits of species that comprise the ecological communities. However, the axes of variation of ecosystem functions are largely unknown, which limits our understanding of how ecosystems respond as a whole to anthropogenic drivers, climate and environmental variability. Here we derive a set of ecosystem functions from a dataset of surface gas exchange measurements across major terrestrial biomes. We find that most of the variability within ecosystem functions (71.8%) is captured by three key axes. The first axis reflects maximum ecosystem productivity and is mostly explained by vegetation structure. The second axis reflects ecosystem water-use strategies and is jointly explained by variation in vegetation height and climate. The third axis, which represents ecosystem carbon-use efficiency, features a gradient related to aridity, and is explained primarily by variation in vegetation structure. We show that two state-of-the-art land surface models reproduce the first and most important axis of ecosystem functions. However, the models tend to simulate more strongly correlated functions than those observed, which limits their ability to accurately predict the full range of responses to environmental changes in carbon, water and energy cycling in terrestrial ecosystems.

High temporal resolution measurements of solar-induced chlorophyll fluorescence (F) and the Photochemical Reflectance Index (PRI) encode vegetation functioning. However, these signals are modulated by time-dependent processes. We tested the applicability of the Singular Spectrum Analysis (SSA) for disentangling fast components (physiology-driven) and slow components (controlled by structural and biochemical properties) from PRI, far-red F (F760), and far-red apparent fluorescence yield (Fy∗760). The proof of concept was developed on spectral and flux time series simulated with the Soil Canopy Observation of Photochemistry and Energy fluxes (SCOPE) model. This allowed the evaluation of SSA decomposition against variables that are independent of physiology or are modified by it. Slow SSA-decomposed components of PRI and Fy∗760 showed high correlations with the reference variables (R2 = 0.97 and 0.96, respectively). Fast SSA-decomposed components of PRI and Fy∗760 were better related to the physiological reference variables than the original signals during periods when leaf area index (LAI) was above 1 m2 m−2. The method was also successfully applied to predict light-use efficiency (LUE) from the fast SSA-decomposed components of PRI (R2 = 0.70) and Fy∗760 (R2 = 0.68) when discarding data modeled with LAI < 1 m2 m−2 and short-wave radiation Rin < 250 W m−2. The method was then tested on data acquired in a Mediterranean grassland. In this case, the fast SSA-decomposed component of apparent LUE∗ showed a stronger correlation with the fast SSA-decomposed component of Fy∗760 (R2 = 0.42) than with original Fy∗760 (R2 = 0.01). SSA-based approach is a promising tool for decoupling physiological information from measurements acquired with automated proximal sensing systems.

Recent advances in remote sensing of solar-induced chlorophyll fluorescence (SIF) have improved the capabilities of monitoring large-scale Gross Primary Productivity (GPP). However, SIF observations are subject to directional effects which can lead to considerable uncertainties in various applications. Practical approaches for normalizing directional SIF observations to nadir viewing, to minimize the directional effects, have not been well studied. Here we developed two practical and physically-solid approaches for removing the directional effects of anisotropic SIF observations: one is based on near-infrared or red reflectance of vegetation (NIRv and Redv), and the other is based on the kernel-driven model with multi-angular SIF measurements. The first approach uses surface reflectance while the second approach directly leverages multi-angular SIF measurements. The performance of the two approaches was evaluated using a dataset of multi-angular measurements of SIF and reflectance collected with a high-resolution field spectrometer over different plant canopies. Results show that the relative mean absolute errors between the normalized nadir SIF and the observed SIF at nadir decrease by 3–6% (far-red) and 6–8% (red) for the first approach, and by 7–13% and 6–11% for the second approach, compared to the original data, respectively. The effectiveness and simplicity of our proposed approaches provide great potential to generate long-term and consistent SIF data records with minimized directional effects.

Nanoparticle assisted drug delivery to the cytoplasm is limited by sequestration of nanoparticles in endosomes. Endosomal escape through polymer-induced membrane destabilization is one of a few well characterized mechanisms to overcome it. Aiming to utilize this method in vivo, it is necessary to understand how modulating the structural and chemical features of the polymer and the presence of proteins in biological fluids can affect this activity. Here, as a model for the endosomal membrane, we use the membrane of red blood cells to evaluate the membrane destabilization ability of a model amphiphilic polymer in the presence of blood plasma using a hemolysis assay. This allows determination of red blood cells membrane permeabilization through the quantification of hemoglobin leakage. Our results showed a strong inhibitory effect of plasma, and that hemolytic activity can be improved by chemical modification of the polymeric micelle, reducing its interaction with plasma proteins. Finally, a second mechanism of pH-induced direct diffusion is proposed and tested using an oil/water partitioning model. These results offer a body of knowledge to improve delivery of drugs across biological membranes using carefully designed polymeric nanocarriers.

Plant functional diversity, defined as the range of plant chemical, physiological and structural properties within plants, is a key component of biodiversity which controls the ecosystem functioning and stability. Monitoring its variations across space and over time is critical in ecological studies. So far, several reflectance-based metrics have been tested to achieve this objective, yielding different degrees of success. Our work aimed at exploring the potential of a novel metric based on far-red sun-induced chlorophyll fluorescence (F760) to map the functional diversity of terrestrial ecosystems. This was achieved exploiting high-resolution images collected over a mixed forest ecosystem with the HyPlant sensor, deployed as an airborne demonstrator of the forthcoming ESA-FLEX satellite. A reference functional diversity map was obtained applying the Rao's Q entropy metric on principal components calculated on key plant functional trait maps retrieved from the hyperspectral reflectance cube. Based on the spectral variation hypothesis, which states that the biodiversity signal is encoded in the spectral heterogeneity, two moving window-based approaches were tested to estimate the functional diversity from continuous spectral data: i) the Rao's Q entropy metric calculated on the normalized difference vegetation index (NDVI) and ii) the coefficient of variation (CV) calculated on hyperspectral reflectance. Finally, a third moving window approach was used to estimate the functional diversity based on F760 heterogeneity quantified through the calculation of the Rao's Q entropy metric. Results showed a strong underestimation of the functional diversity using the Rao's Q index based on NDVI and the CV of reflectance. In both cases, a weak correlation was found against the reference functional diversity map (r2 = 0.05, p < .001 and r2 = 0.04, p < .001, respectively). Conversely, the Rao's Q index calculated on F760 revealed similar patterns as the ones observed in the

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Estimating gross primary production (GPP), the gross uptake of CO2 by vegetation, is a fundamental prerequisite for understanding and quantifying the terrestrial carbon cycle. Over the last decade, multiple approaches have been developed to derive spatiotemporal dynamics of GPP combining in situ observations and remote sensing data using machine learning techniques or semiempirical models. However, no high spatial resolution GPP product exists so far that is derived entirely from satellite-based remote sensing data. Sentinel-2 satellites are expected to open new opportunities to analyze ecosystem processes with spectral bands chosen to study vegetation between 10- and 20-m spatial resolutions with five-day revisit frequency. Of particular relevance is the availability of red-edge bands that are suitable for deriving estimates of canopy chlorophyll content that are expected to be much better than any previous global mission. Here, we analyzed whether red-edge-based and near-infrared-based vegetation indices (VIs) or machine learning techniques that consider VIs, all spectral bands, and their nonlinear interactions could predict daily GPP derived from 58 eddy covariance sites. Using linear regressions based on classic VIs, including near-infrared reflectance of vegetation (NIRv), we achieved prediction powers of $R^{2}_{\mathrm{10-fold}} = 0.51$ and an $RMSE_{\mathrm{10-fold}} = 2.95 $ [ $\mu \rm {mol \ CO_{2} m^{-2}s^{-1}}$ ] in a 10-fold cross validation. Chlorophyll index red (CIR) and the novel kernel NDVI (kNVDI) achieved significantly higher prediction powers of around $R^{2}_{\mathrm{10-fold}} \approx 0.61$ and $RMSE_{\mathrm{10-fold}} \approx 2.57$ [ $\mu \rm {mol \ CO_{2} m^{-2}s^{-1}}$ ]. Using all spectral bands and VIs jointly in a machine learning prediction framework allowed us to predict GPP with $R^{2}_{\mathrm{10-fold}} = 0.71$ and $RMSE_{\mathrm{10-fold}} = 2.68$ [ $\mu \rm {mol \ CO_{2} m^{-2}s^{-1}}$ ]. Despite the high-power prediction when machin

In a context of accelerated human-induced biodiversity loss, remote sensing (RS) is emerging as a promising tool to map plant biodiversity from space. Proposed approaches often rely on the Spectral Variation Hypothesis (SVH), linking the heterogeneity of terrestrial vegetation to the variability of the spectroradiometric signals. Yet, due to observational limitations, the SVH has been insufficiently tested, remaining unclear which metrics, methods, and sensors could provide the most reliable estimates of plant biodiversity. Here we assessed the potential of RS to infer plant biodiversity using radiative transfer simulations and inversion. We focused specifically on “functional diversity,” which represents the spatial variability in plant functional traits. First, we simulated vegetation communities and evaluated the information content of different functional diversity metrics (FDMs) derived from their optical reflectance factors (R) or the corresponding vegetation “optical traits,” estimated via radiative transfer model inversion. Second, we assessed the effect of the spatial resolution, the spectral characteristics of the sensor, and signal noise on the relationships between FDMs derived from field and remote sensing datasets. Finally, we evaluated the plausibility of the simulations using Sentinel-2 (multispectral, 10 m pixel) and DESIS (hyperspectral, 30 m pixel) imagery acquired over sites of the Functional Significance of Forest Biodiversity in Europe (FunDivEUROPE) network. We demonstrate that functional diversity can be inferred both by reflectance and optical traits. However, not all the FDMs tested were suited for assessing plant functional diversity from RS. Rao's Q index, functional dispersion, and functional richness were the best-performing metrics. Furthermore, we demonstrated that spatial resolution is the most limiting RS feature. In agreement with simulations, Sentinel-2 imagery provided better estimates of plant diversity than DESIS, despite the c

The input of liquid water to terrestrial ecosystems is composed of rain and non-rainfall water (NRW). The latter comprises dew, fog, and the adsorption of atmospheric vapor on soil particle surfaces. Although NRW inputs can be relevant to support ecosystem functioning in seasonally dry ecosystems, they are understudied, being relatively small, and therefore hard to measure. In this study, we apply a partitioning routine focusing on NRW inputs over 1 year of data from large, high-precision weighing lysimeters at a semi-arid Mediterranean site. NRW inputs occur for at least 3 h on 297 d (81 % of the year), with a mean diel duration of 6 h. They reflect a pronounced seasonality as modulated by environmental conditions (i.e., temperature and net radiation). During the wet season, both dew and fog dominate NRW, while during the dry season it is mostly the soil adsorption of atmospheric water vapor. Although NRW contributes only 7.4 % to the annual water input, NRW is the only water input to the ecosystem during 15 weeks, mainly in the dry season. Benefitting from the comprehensive set of measurements at our experimental site, we show that our findings are in line with (i) independent measurements and (ii) independent model simulations forced with (near-) surface energy and moisture measurements. Furthermore, we discuss the simultaneous occurrence of soil vapor adsorption and negative eddy-covariance-derived latent heat fluxes. This study shows that NRW inputs can be reliably detected through high-resolution weighing lysimeters and a few additional measurements. Their main occurrence during nighttime underlines the necessity to consider ecosystem water fluxes at a high temporal resolution and with 24 h coverage.

Vegetation plays a vital role in the Earth system by sequestering carbon, producing food and oxygen, and providing evaporative cooling. Vegetation productivity extremes have multi-faceted implications, for example on crop yields or the atmospheric CO2 concentration. Here, we focus on productivity extremes as possible impacts of coinciding, potentially extreme hydrometeorological anomalies. Using monthly global satellite-based Sun-induced chlorophyll fluorescence data as a proxy for vegetation productivity from 2007–2015, we show that vegetation productivity extremes are related to hydrometeorological hazards as characterized through ERA5-Land reanalysis data in approximately 50 % of our global study area. For the latter, we are considering sufficiently vegetated and cloud-free regions, and we refer to hydrometeorological hazards as water- or energy-related extremes inducing productivity extremes. The relevance of the different hazard types varies in space; temperature-related hazards dominate at higher latitudes with cold spells contributing to productivity minima and heat waves supporting productivity maxima, while water-related hazards are relevant in the (sub-)tropics with droughts being associated with productivity minima and wet spells with the maxima. Alongside single hazards compound events such as joint droughts and heat waves or joint wet and cold spells also play a role, particularly in dry and hot regions. Further, we detect regions where energy control transitions to water control between maxima and minima of vegetation productivity. Therefore, these areas represent hotspots of land–atmosphere coupling where vegetation efficiently translates soil moisture dynamics into surface fluxes such that the land affects near-surface weather. Overall, our results contribute to pinpointing how potential future changes in temperature and precipitation could propagate to shifting vegetation productivity extremes and related ecosystem services.

X-linked chronic granulomatous disease is a rare disease caused by mutations in the CYBB gene. While more extensive knowledge is available on genetics, pathogenesis, and possible therapeutic options, mitochondrial activity and its implications on patient monitoring are still not well-characterized. We have developed a novel protocol to study mitochondrial activity on whole blood of XCGD patients before and after transplantation, as well as on XCGD carriers. Here we present results of these analyses and of the restoration of mitochondrial activity in hyperinflamed X-linked Chronic Granulomatous Disease after hematopoietic stem cell transplantation. Moreover, we show a strong direct correlation between mitochondrial activity, chimerism, and DHR monitored before and after transplantation and in XCGD carriers. In conclusion, based on these findings, we suggest testing this new ready-to-use marker to better characterize patients before and after treatment and to investigate disease expression in carriers.

The sensitivity of photosynthesis to environmental changes is essential for understanding carbon cycle responses to global climate change and for the development of modeling approaches that explains its spatial and temporal variability. We collected a large variety of published sensitivity functions of gross primary productivity (GPP) to different forcing variables to assess the response of GPP to environmental factors. These include the responses of GPP to temperature; vapor pressure deficit, some of which include the response to atmospheric CO2 concentrations; soil water availability (W); light intensity; and cloudiness. These functions were combined in a full factorial light use efficiency (LUE) model structure, leading to a collection of 5600 distinct LUE models. Each model was optimized against daily GPP and evapotranspiration fluxes from 196 FLUXNET sites and ranked across sites based on a bootstrap approach. The GPP sensitivity to each environmental factor, including CO2 fertilization, was shown to be significant, and that none of the previously published model structures performed as well as the best model selected. From daily and weekly to monthly scales, the best model's median Nash-Sutcliffe model efficiency across sites was 0.73, 0.79 and 0.82, respectively, but poorer at annual scales (0.23), emphasizing the common limitation of current models in describing the interannual variability of GPP. Although the best global model did not match the local best model at each site, the selection was robust across ecosystem types. The contribution of light saturation and cloudiness to GPP was observed across all biomes (from 23% to 43%). Temperature and W dominates GPP and LUE but responses of GPP to temperature and W are lagged in cold and arid ecosystems, respectively. The findings of this study provide a foundation towards more robust LUE-based estimates of global GPP and may provide a benchmark for other empirical GPP products.

The upcoming Fluorescence Explorer (FLEX) mission will provide sun-induced fluorescence (SIF) products at unprecedented spatial resolution. Thus, accurate calibration and validation (cal/val) of these products are key to guarantee robust SIF estimates for the assessment and quantification of photosynthetic processes. In this study, we address one specific component of the uncertainty budget related to SIF retrieval: the spatial representativeness of in situ SIF observations compared to medium-resolution SIF products (e.g., 300 m pixel size). Here, we propose an approach to evaluate an optimal sampling strategy to characterise the spatial representativeness of in situ SIF observations based on high-spatial-resolution SIF data. This approach was applied for demonstration purposes to two agricultural areas that have been extensively characterized with a HyPlant airborne imaging spectrometer in recent years. First, we determined the spatial representativeness of an increasing number of sampling points with respect to a reference area (either monocultural crop fields or hypothetical FLEX pixels characterised by different land cover types). Then, we compared different sampling approaches to determine which strategy provided the most representative reference data for a given area. Results show that between 3 and 13.5 sampling points are needed to characterise the average SIF value of both monocultural fields and hypothetical FLEX pixels of the agricultural areas considered in this study. The number of sampling points tends to increase with the standard deviation of SIF of the reference area, as well as with the number of land cover classes in a FLEX pixel, even if the increase is not always statistically significant. This study contributes to guiding cal/val activities for the upcoming FLEX mission, providing useful insights for the selection of the validation site network and particularly for the definition of the best sampling scheme for each site.

Sun-Induced fluorescence at 760 nm (F760) is increasingly being used to predict gross primary production (GPP) through light use efficiency (LUE) modeling, even though the mechanistic processes that link the two are not well understood. We analyzed the effect of nitrogen (N) and phosphorous (P) availability on the processes that link GPP and F760 in a Mediterranean grassland manipulated with nutrient addition. To do so, we used a combination of process-based modeling with Soil-Canopy Observation of Photosynthesis and Energy (SCOPE), and statistical analyses such as path modeling. With this study, we uncover the mechanisms that link the fertilization-driven changes in canopy nitrogen concentration (N%) to the observed changes in F760 and GPP. N addition changed plant community structure and increased canopy chlorophyll content, which jointly led to changes in photosynthetic active radiation (APAR), ultimately affecting both GPP and F760. Changes in the abundance of graminoids, (%graminoids) driven by N addition led to changes in structural properties of the canopy such as leaf angle distribution, that ultimately influenced observed F760 by controlling the escape probability of F760 (Fesc). In particular, we found a change in GPP-F760 relationship between the first and the second year of the experiment that was largely driven by the effect of plant type composition on Fesc, whose best predictor is %graminoids. The P addition led to a statistically significant increase on light use efficiency of fluorescence emission (LUEf), in particular in plots also with N addition, consistent with leaf level studies. The N addition induced changes in the biophysical properties of the canopy that led to a trade-off between surface temperature (Ts), which decreased, and F760 at leaf scale (F760leaf,fw), which increased. We found that Ts is an important predictor of the light use efficiency of photosynthesis, indicating the importance of Ts in LUE modeling approaches to predict GPP

The most recent efforts to provide remote sensing (RS) estimates of plant function rely on the combination of Radiative Transfer Models (RTM) and Soil-Vegetation-Atmosphere Transfer (SVAT) models, such as the Soil-Canopy Observation Photosynthesis and Energy fluxes (SCOPE) model. In this work we used ground spectroradiometric and chamber-based CO2 flux measurements in a nutrient manipulated Mediterranean grassland in order to: 1) develop a multiple-constraint inversion approach of SCOPE able to retrieve vegetation biochemical, structural as well as key functional traits, such as chlorophyll concentration (Cab), leaf area index (LAI), maximum carboxylation rate (Vcmax) and the Ball-Berry sensitivity parameter (m); and 2) compare the potential of the of gross primary production (GPP) and sun-induced fluorescence (SIF), together with up-welling Thermal Infrared (TIR) radiance and optical reflectance factors (RF), to estimate such parameters. The performance of the proposed inversion method as well as of the different sets of constraints was assessed with contemporary measurements of water and heat fluxes and leaf nitrogen content, using pattern-oriented model evaluation. The multiple-constraint inversion approach proposed together with the combination of optical RF and diel GPP and TIR data provided reliable estimates of parameters, and improved predicted water and heat fluxes. The addition of SIF to this scheme slightly improved the estimation of m. Parameter estimates were coherent with the variability imposed by the fertilization and the seasonality of the grassland. Results revealed that fertilization had an impact on Vcmax, while no significant differences were found for m. The combination of RF, SIF and diel TIR data weakly constrained functional traits. Approaches not including GPP failed to estimate LAI; however GPP overestimated Cab in the dry period. These problems might be related to the presence of high fractions of senescent leaves in the grassland. The pr

A major international effort has been made to monitor sun-induced chlorophyll fluorescence (SIF) from space as a proxy for the photosynthetic activity of terrestrial vegetation. However, the effect of spatial heterogeneity on the SIF retrievals from canopy radiance derived from images with medium and low spatial resolution remains uncharacterised. In images from forest and agricultural landscapes, the background comprises a mixture of soil and understory and can generate confounding effects that limit the interpretation of the SIF at the canopy level. This paper aims to improve the understanding of SIF from coarse spatial resolutions in heterogeneous canopies by considering the separated contribution of tree crowns, understory and background components, using a modified version of the FluorFLIGHT radiative transfer model (RTM). The new model is compared with others through the RAMI model intercomparison framework and is validated with airborne data. The airborne campaign includes high-resolution data collected over a tree-grass ecosystem with the HyPlant imaging spectrometer within the FLuorescence EXplorer (FLEX) preparatory missions. Field data measurements were collected from plots with a varying fraction of tree and understory vegetation cover. The relationship between airborne SIF calculated from pure tree crowns and aggregated pixels shows the effect of the understory at different resolutions. For a pixel size smaller than the mean crown size, the impact of the background was low (R² > 0.99; NRMSE < 0.01). By contrast, for a pixel size larger than the crown size, the goodness of fit decreased (R² < 0.6; NRMSE > 0.2). This study demonstrates that using a 3D RTM model improves the calculation of SIF significantly (R² = 0.83, RMSE = 0.03 mW m⁻² sr⁻¹ nm⁻¹) when the specific contribution of the soil and understory layers are accounted for, in comparison with the SIF calculated from mixed pixels that considers only one layer as background (R² = 0.4, RMSE = 0.28 mW m

Im Rahmen einer Langzeitstudie mit wägbaren Präzisionslysimetern konzentrieren wir uns auf die Unterscheidung und Quantifizierung unterschiedlicher Wasserflüsse aus der Atmosphäre in den Boden: Neben Regen zählen dazu Taubildung, Nebelniederschlag und Bodenwasseradsorption, wobei die drei letzteren als nicht-Regen Wassereintrag (NRWI) zusammengefasst werden. Um diese Prozesse in den Gewichtsänderungen der Lysimeter zu unterscheiden, wurden meteorologische, bodenhydrologische und radiometrische Daten genutzt. Unsere Ergebnisse zeigen, dass im Zeitraum 2018 - 2020 in 72.8 % der Nächte Wasser als NRWI auf der Oberfläche und im Boden kondensiert ist. Der Gesamteintrag beträgt im Mittel 43.5 mm, was 8.1% des mittleren jährlichen Inputs darstellt. Während zwischen Oktober und April Wasser hauptsächlich als Tau kondensiert, ist Bodenwasseradsorption im Mediterranen Hochsommer zwischen Juli und September der einzige NRWI Wassereintrag.

Plant transpiration links physiological responses of vegetation to water supply and demand with hydrological, energy, and carbon budgets at the land-atmosphere interface. However, despite being the main land evaporative flux at the global scale, transpiration and its response to environmental drivers are currently not well constrained by observations. Here we introduce the first global compilation of whole-plant transpiration data from sap flow measurements (SAPFLUXNET, https://sapfluxnet.creaf.cat/, last access: 8 June 2021). We harmonized and quality-controlled individual datasets supplied by contributors worldwide in a semi-automatic data workflow implemented in the R programming language. Datasets include sub-daily time series of sap flow and hydrometeorological drivers for one or more growing seasons, as well as metadata on the stand characteristics, plant attributes, and technical details of the measurements. SAPFLUXNET contains 202 globally distributed datasets with sap flow time series for 2714 plants, mostly trees, of 174 species. SAPFLUXNET has a broad bioclimatic coverage, with woodland/shrubland and temperate forest biomes especially well represented (80% of the datasets). The measurements cover a wide variety of stand structural characteristics and plant sizes. The datasets encompass the period between 1995 and 2018, with 50% of the datasets being at least 3 years long. Accompanying radiation and vapour pressure deficit data are available for most of the datasets, while on-site soil water content is available for 56% of the datasets. Many datasets contain data for species that make up 90% or more of the total stand basal area, allowing the estimation of stand transpiration in diverse ecological settings. SAPFLUXNET adds to existing plant trait datasets, ecosystem flux networks, and remote sensing products to help increase our understanding of plant water use, plant responses to drought, and ecohydrological processes. SAPFLUXNET version 0.1.5 is freely a

Understanding the dependencies of the terrestrial carbon and water cycle with meteorological conditions is a prerequisite to anticipate their behaviour under climate change conditions. However, terrestrial ecosystems and the atmosphere interact via a multitude of variables across temporal and spatial scales. Additionally these interactions might differ among vegetation types or climatic regions. Today, novel algorithms aim to disentangle the causal structure behind such interactions from empirical data. The estimated causal structures can be interpreted as networks, where nodes represent relevant meteorological variables or land-surface fluxes and the links represent the dependencies among them (possibly including time lags and link strength). Here we derived causal networks for different seasons at 119 eddy covariance flux tower observations in the FLUXNET network. We show that the networks of biosphere–atmosphere interactions are strongly shaped by meteorological conditions. For example, we find that temperate and high-latitude ecosystems during peak productivity exhibit biosphere–atmosphere interaction networks very similar to tropical forests. In times of anomalous conditions like droughts though, both ecosystems behave more like typical Mediterranean ecosystems during their dry season. Our results demonstrate that ecosystems from different climate zones or vegetation types have similar biosphere–atmosphere interactions if their meteorological conditions are similar. We anticipate our analysis to foster the use of network approaches, as they allow for a more comprehensive understanding of the state of ecosystem functioning. Long-term or even irreversible changes in network structure are rare and thus can be indicators of fundamental functional ecosystem shifts.

The leaf economics spectrum(1,2) and the global spectrum of plant forms and functions(3) revealed fundamental axes of variation in plant traits, which represent different ecological strategies that are shaped by the evolutionary development of plant species(2). Ecosystem functions depend on environmental conditions and the traits of species that comprise the ecological communities(4). However, the axes of variation of ecosystem functions are largely unknown, which limits our understanding of how ecosystems respond as a whole to anthropogenic drivers, climate and environmental variability(4,5). Here we derive a set of ecosystem functions(6) from a dataset of surface gas exchange measurements across major terrestrial biomes. We find that most of the variability within ecosystem functions (71.8%) is captured by three key axes. The first axis reflects maximum ecosystem productivity and is mostly explained by vegetation structure. The second axis reflects ecosystem water-use strategies and is jointly explained by variation in vegetation height and climate. The third axis, which represents ecosystem carbon-use efficiency, features a gradient related to aridity, and is explained primarily by variation in vegetation structure. We show that two state-of-the-art land surface models reproduce the first and most important axis of ecosystem functions. However, the models tend to simulate more strongly correlated functions than those observed, which limits their ability to accurately predict the full range of responses to environmental changes in carbon, water and energy cycling in terrestrial ecosystems(7,8).

Background Allogeneic hematopoietic stem-cell transplantation is the standard of care for Hurler syndrome (mucopolysaccharidosis type I, Hurler variant [MPSIH]). However, this treatment is only partially curative and is associated with complications. Methods We are conducting an ongoing study involving eight children with MPSIH. At enrollment, the children lacked a suitable allogeneic donor and had a Developmental Quotient or Intelligence Quotient score above 70 (i.e., none had moderate or severe cognitive impairment). The children received autologous hematopoietic stem and progenitor cells (HSPCs) transduced ex vivo with an alpha-L-iduronidase (IDUA)-encoding lentiviral vector after myeloablative conditioning. Safety and correction of blood IDUA activity up to supraphysiologic levels were the primary end points. Clearance of lysosomal storage material as well as skeletal and neurophysiological development were assessed as secondary and exploratory end points. The planned duration of the study is 5 years. Results We now report interim results. The children's mean (+/- SD) age at the time of HSPC gene therapy was 1.9 +/- 0.5 years. At a median follow-up of 2.10 years, the procedure had a safety profile similar to that known for autologous hematopoietic stem-cell transplantation. All the patients showed prompt and sustained engraftment of gene-corrected cells and had supraphysiologic blood IDUA activity within a month, which was maintained up to the latest follow-up. Urinary glycosaminoglycan (GAG) excretion decreased steeply, reaching normal levels at 12 months in four of five patients who could be evaluated. Previously undetectable levels of IDUA activity in the cerebrospinal fluid became detectable after gene therapy and were associated with local clearance of GAGs. Patients showed stable cognitive performance, stable motor skills corresponding to continued motor development, improved or stable findings on magnetic resonance imaging of the brain and spine, reduced

Sun induced fluorescence at 760 nm (F 760 ) has shown to provide a valid approach to quantify gross primary production (GPP) at various scales, however the relationship between GPP and F 760 is influenced by the escape probability of fluorescence (Fesc), a variable which is not still fully understood. Combining radiative transfer modelling approaches, by means of the SCOPE model, and a data driven methodology based on variable selection methods we identify the predictors of Fesc, focusing on the effect of functional and structural traits. We show that Fesc is mainly predicted by structural variables such as fraction of grasses and near infrared reflectance. Building on the analysis of the predictor of Fesc, LUE p and LUE f we present a semi-empirical model formulation based only on optical data that significantly improves the GPP prediction.

The SCOPE model embeds the state of art for coupling soil vegetation atmosphere transfer (SVAT) and radiative transfer models (RTM). For that reason the FLuorescence EXplorer (FLEX) mission selected this model to derive vegetation properties through inversion. However inverse problem is often ill-posed, providing equally likely solutions and hence inflating the uncertainty of the retrieved parameters. In this work we test the use of different priors based on ancillary measurements and literature to support multiple-constrain inversion of SCOPE. Results show that prior information on the relationships between variables such as leaf chlorophyll content (Cab), leaf carotenoids content (Cca), leaf water content (Cw) and/or maximum carboxylation rate (Vcmax) reduce inversion uncertainties and overfitting, and should be sampled/estimated together with optical data.