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21.
The antisense oligonucleotide Nusinersen has been recently licensed to treat spinal muscular atrophy (SMA). Since SMA type 3 is characterized by variable phenotype and milder progression, biomarkers of early treatment response are urgently needed. We investigated the cerebrospinal fluid (CSF) concentration of neurofilaments in SMA type 3 patients treated with Nusinersen as a potential biomarker of treatment efficacy. The concentration of phosphorylated neurofilaments heavy chain (pNfH) and light chain (NfL) in the CSF of SMA type 3 patients was evaluated before and after six months since the first Nusinersen administration, performed with commercially available enzyme-linked immunosorbent assay (ELISA) kits. Clinical evaluation of SMA patients was performed with standardized motor function scales. Baseline neurofilament levels in patients were comparable to controls, but significantly decreased after six months of treatment, while motor functions were only marginally ameliorated. No significant correlation was observed between the change in motor functions and that of neurofilaments over time. The reduction of neurofilament levels suggests a possible early biochemical effect of treatment on axonal degeneration, which may precede changes in motor performance. Our study mandates further investigations to assess neurofilaments as a marker of treatment response.  相似文献   
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Monitoring ecosystem functions in forests is a priority in a climate change scenario, as climate‐induced events may initially alter the functions more than slow‐changing attributes, such as biomass. The ecosystem functional properties (EFPs) are quantities that characterize key ecosystem processes. They can be derived by point observations of gas and energy exchanges between the ecosystems and the atmosphere that are collected globally at FLUXNET flux tower sites and upscaled at ecosystem level. The properties here considered describe the ability of ecosystems to optimize the use of resources for carbon uptake. They represent functional forest information, are dependent on environmental drivers, linked to leaf traits and forest structure, and influenced by climate change effects. The ability of vegetation optical depth (VOD) to provide forest functional information is investigated using 2011–2014 satellite data collected by the Soil Moisture and Ocean Salinity mission and using the EFPs as reference dataset. Tropical forests in Africa and South America were analyzed, also according to ecological homogeneous units. VOD jointly with water deficit information explained 93% and 87% of the yearly variability in both flux upscaled maximum gross primary productivity and light use efficiency functional properties, in Africa and South America forests respectively. Maps of the retrieved properties evidenced changes in forest functional responses linked to anomalous climate‐induced events during the study period. The findings indicate that VOD can support the flux upscaling process in the tropical range, affected by high uncertainty, and the detection of forest anomalous functional responses. Preliminary temporal analysis of VOD and EFP signals showed fine‐grained variability in periodicity, in signal dephasing, and in the strength of the relationships. In selected drier forest types, these satellite data could also support the monitoring of functional dynamics.  相似文献   
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There is growing international interest in better managing soils to increase soil organic carbon (SOC) content to contribute to climate change mitigation, to enhance resilience to climate change and to underpin food security, through initiatives such as international ‘4p1000’ initiative and the FAO's Global assessment of SOC sequestration potential (GSOCseq) programme. Since SOC content of soils cannot be easily measured, a key barrier to implementing programmes to increase SOC at large scale, is the need for credible and reliable measurement/monitoring, reporting and verification (MRV) platforms, both for national reporting and for emissions trading. Without such platforms, investments could be considered risky. In this paper, we review methods and challenges of measuring SOC change directly in soils, before examining some recent novel developments that show promise for quantifying SOC. We describe how repeat soil surveys are used to estimate changes in SOC over time, and how long‐term experiments and space‐for‐time substitution sites can serve as sources of knowledge and can be used to test models, and as potential benchmark sites in global frameworks to estimate SOC change. We briefly consider models that can be used to simulate and project change in SOC and examine the MRV platforms for SOC change already in use in various countries/regions. In the final section, we bring together the various components described in this review, to describe a new vision for a global framework for MRV of SOC change, to support national and international initiatives seeking to effect change in the way we manage our soils.  相似文献   
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Environmental Biology of Fishes - This study evaluated how the plasma steroid hormones testosterone (T) and 17β-estradiol (E2) are related to follicular development in regenerating females of...  相似文献   
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Chloroplasts of land plants characteristically contain grana, cylindrical stacks of thylakoid membranes. A granum consists of a core of appressed membranes, two stroma-exposed end membranes, and margins, which connect pairs of grana membranes at their lumenal sides. Multiple forces contribute to grana stacking, but it is not known how the extreme curvature at margins is generated and maintained. We report the identification of the CURVATURE THYLAKOID1 (CURT1) protein family, conserved in plants and cyanobacteria. The four Arabidopsis thaliana CURT1 proteins (CURT1A, B, C, and D) oligomerize and are highly enriched at grana margins. Grana architecture is correlated with the CURT1 protein level, ranging from flat lobe-like thylakoids with considerably fewer grana margins in plants without CURT1 proteins to an increased number of membrane layers (and margins) in grana at the expense of grana diameter in overexpressors of CURT1A. The endogenous CURT1 protein in the cyanobacterium Synechocystis sp PCC6803 can be partially replaced by its Arabidopsis counterpart, indicating that the function of CURT1 proteins is evolutionary conserved. In vitro, Arabidopsis CURT1A proteins oligomerize and induce tubulation of liposomes, implying that CURT1 proteins suffice to induce membrane curvature. We therefore propose that CURT1 proteins modify thylakoid architecture by inducing membrane curvature at grana margins.  相似文献   
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Abstract

The discover and morphological characters of a Dematiaceous Hyphomycetes are described. The material, studied under a morphological point of view directly collected on the natural substratum, allows us to establish the appartenance to the genus Chaetopsina. Since this species does not appeare described till now, the Authors propose for it the nomenclature ivoriensis as a new species.  相似文献   
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