How do geomorphic characteristics affect the source of tree water uptake in restored river floodplains?

Alpine rivers and their floodplains have been highly modified by human activities during the last decades. River restoration projects aim to counteract these negative impacts and to restore ecosystem services provided by riparian habitats. We studied two recently restored river sites in the Ahr/Aurino and Mareit/Mareta Rivers (Italian Alps) to investigate how geomorphic conditions, soil moisture, and groundwater level affect the source of water used by grey alder (Alnus incana (L.) Moench). We compared the isotopic composition (δ2H) of tree sap at different locations (low terraces formed during bed incision and recent floodplains formed after restoration) with that of potential water sources, that is, groundwater, soil water, and rainfall. The monthly variation in the isotopic composition of rainfall was reflected in both shallow and deeper soil water, as well as in the isotopic composition of sap. The redistribution of precipitation and groundwater in the soil differed between the post-restoration floodplain sites and the post-incision terraces, leading to a different relation between the sap water, soil water, and groundwater isotopic composition. The results show that transpiration of A. incana trees growing on recent floodplains is mostly supported by stream-fed soil water, whereas trees growing on terraces mainly use precipitation-fed soil water. These marked, morphology-related differences in the source of transpiration water of grey alder highlight how channel degradation still affects the ecohydrological processes in Alpine fluvial corridors. Nonetheless, large restoration interventions—in terms of channel widening—can enable the self-formation of new floodplain areas characterized by stream water-fed riparian ecosystems

Novel mutations in DNA2 associated with myopathy and mtDNA instability

The maintenance of mitochondrial DNA (mtDNA) relies on proteins encoded by nuclear genes. Mutations in their coding sequences result in heterogenous clinical presentations featuring mtDNA instability in affected tissues. DNA2 is a multi-catalytic protein involved in the removal of single strand DNA during mtDNA replication or Long Patch Base Excision Repair pathway. We have previously described DNA2 mutations in adult patients affected with familial and sporadic forms of mitochondrial myopathy. Here we describe four novel probands presenting with limb weakness associated with novel DNA2 molecular defects. Biochemical assays were established to investigate the functional effects of these variants

Novel mutations in DNA2 associated with myopathy and mtDNA instability

The maintenance of mitochondrial DNA (mtDNA) relies on proteins encoded by nuclear genes. Mutations in their coding sequences result in heterogenous clinical presentations featuring mtDNA instability in affected tissues. DNA2 is a multi-catalytic protein involved in the removal of single strand DNA during mtDNA replication or Long Patch Base Excision Repair pathway. We have previously described DNA2 mutations in adult patients affected with familial and sporadic forms of mitochondrial myopathy. Here we describe four novel probands presenting with limb weakness associated with novel DNA2 molecular defects. Biochemical assays were established to investigate the functional effects of these variants

Sprachwissenschaft im III. Reich : ein erster Überblick

Der Themenbereich "Sprachwissenschaft im III. Reich" muß als ungewöhnlich wenig erforscht gelten. Linguisten haben sich daran nahezu überhaupt nicht beteiligt. Die wenigen Kenntnisse, die wir bislang über diesen Bereich haben, verdanken wir vorwiegend Zeitgeschichtsforschern oder Kulturwissenschaftlern, in deren Arbeiten sie allerdings mehr versteckt als veröffentlicht sind. Im Vergleich zu den Kenntnissen, die Sprachwissenschaftler über die Geschichte ihres Fachs im 19. Jahrhundert oder ausländische Forschungsrichtungen wie Strukturalismus und Sprechakttheorie in der Regel haben, ist überdies das Wissen über die Linguistik im Deutschland des III. Reichs auffällig gering

Conversion of the BASE Prion Strain into the BSE Strain: The Origin of BSE?

Atypical neuropathological and molecular phenotypes of bovine spongiform encephalopathy (BSE) have recently been identified in different countries. One of these phenotypes, named bovine “amyloidotic” spongiform encephalopathy (BASE), differs from classical BSE for the occurrence of a distinct type of the disease-associated prion protein (PrP), termed PrP(Sc), and the presence of PrP amyloid plaques. Here, we show that the agents responsible for BSE and BASE possess different biological properties upon transmission to transgenic mice expressing bovine PrP and inbred lines of nontransgenic mice. Strikingly, serial passages of the BASE strain to nontransgenic mice induced a neuropathological and molecular disease phenotype indistinguishable from that of BSE-infected mice. The existence of more than one agent associated with prion disease in cattle and the ability of the BASE strain to convert into the BSE strain may have important implications with respect to the origin of BSE and spongiform encephalopathies in other species, including humans

E-commerce adoption by SMEs in developing countries: evidence from Indonesia

This study aims to provide an overview of e-commerce adoption by SMEs in developing countries and, in particular, the extent of the adoption of e-commerce by Indonesian SMEs. It identifies the e-commerce benefits realized by these SMEs and investigates the relationship between the levels of e-commerce adoption and the benefits thus realized. The study was motivated by the limited studies related to e-commerce adoption by SMEs, especially in developing countries. In addition, it seems that most e-commerce studies are focused more on upstream issues: to see the factors that facilitate, or barriers faced regarding e-commerce adoption, rather than downstream issues: to see post-adoption benefits. This certainly limits our understanding about e-commerce adoption by SMEs in developing countries, as well as the post-adoption benefits of e-commerce. Indonesia was chosen as the place in which to conduct the study. A survey of 292 SMEs shows that the majority of them are still at an early stage in their adoption of e-commerce. Their use of e-commerce is dominated by marketing and purchasing and procurement activities. “Extending market reach”, “increased sales”, “improved external communication”, “improved company image”, “improved speed of processing”, and “increased employee productivity” are reported as the top six e-commerce benefits perceived by these SMEs. This study also shows that SMEs at the higher level of e-commerce adoption experience greater e-commerce benefits than those at other levels of adoption

Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy

The amyloidotic form of bovine spongiform encephalopathy (BSE) termed BASE is caused by a prion strain whose biological properties differ from those of typical BSE, resulting in a clinically and pathologically distinct phenotype. Whether peripheral tissues of BASE-affected cattle contain infectivity is unknown. This is a critical issue since the BASE prion is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible. We carried out bioassays in transgenic mice overexpressing bovine PrP (Tgbov XV) and found infectivity in a variety of skeletal muscles from cattle with natural and experimental BASE. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases (∼70% versus ∼10%, respectively). This difference was likely related to different prion titers, possibly due to different stages of disease in the two conditions, i.e. terminal stage in experimental BASE and pre-symptomatic stage in natural BASE. The neuropathological phenotype and PrPres type were consistent in all affected mice and matched those of Tgbov XV mice infected with brain homogenate from natural BASE. The immunohistochemical analysis of skeletal muscles from cattle with natural and experimental BASE showed the presence of abnormal prion protein deposits within muscle fibers. Conversely, Tgbov XV mice challenged with lymphoid tissue and kidney from natural and experimental BASE did not develop disease. The novel information on the neuromuscular tropism of the BASE strain, efficiently overcoming species barriers, underlines the relevance of maintaining an active surveillance

Immunopurification of Pathological Prion Protein Aggregates

Background: Prion diseases are fatal neurodegenerative disorders that can arise sporadically, be genetically inherited or acquired through infection. The key event in these diseases is misfolding of the cellular prion protein (PrP) into a pathogenic isoform that is rich in β-sheet structure. This conformational change may result in the formation of PrP, the prion isoform of PrP, which propagates itself by imprinting its aberrant conformation onto PrP molecules. A great deal of effort has been devoted to developing protocols for purifying PrP for structural studies, and testing its biological properties. Most procedures rely on protease digestion, allowing efficient purification of PrP27-30, the protease-resistant core of PrP. However, protease treatment cannot be used to isolate abnormal forms of PrP lacking conventional protease resistance, such as those found in several genetic and atypical sporadic cases. Principal Findings: We developed a method for purifying pathological PrP molecules based on sequential centrifugation and immunoprecipitation with a monoclonal antibody selective for aggregated PrP. With this procedure we purified full-length PrP and mutant PrP aggregates at electrophoretic homogeneity. PrP purified from prion-infected mice was able to seed misfolding of PrP in a protein misfolding cyclic amplification reaction, and mutant PrP aggregates from transgenic mice were toxic to cultured neurons. Significance: The immunopurification protocol described here isolates biologically active forms of aggregated PrP. These preparations may be useful for investigating the structural and chemico-physical properties of infectious and neurotoxic PrP aggregates

Data-driven staging of genetic frontotemporal dementia using multi-modal MRI

Frontotemporal dementia in genetic forms is highly heterogeneous and begins many years to prior symptom onset, complicating disease understanding and treatment development. Unifying methods to stage the disease during both the presymptomatic and symptomatic phases are needed for the development of clinical trials outcomes. Here we used the contrastive trajectory inference (cTI), an unsupervised machine learning algorithm that analyzes temporal patterns in high-dimensional large-scale population datasets to obtain individual scores of disease stage. We used cross-sectional MRI data (gray matter density, T1/T2 ratio as a proxy for myelin content, resting-state functional amplitude, gray matter fractional anisotropy, and mean diffusivity) from 383 gene carriers (269 presymptomatic and 115 symptomatic) and a control group of 253 noncarriers in the Genetic Frontotemporal Dementia Initiative. We compared the cTI-obtained disease scores to the estimated years to onset (age-mean age of onset in relatives), clinical, and neuropsychological test scores. The cTI based disease scores were correlated with all clinical and neuropsychological tests (measuring behavioral symptoms, attention, memory, language, and executive functions), with the highest contribution coming from mean diffusivity. Mean cTI scores were higher in the presymptomatic carriers than controls, indicating that the method may capture subtle pre-dementia cerebral changes, although this change was not replicated in a subset of subjects with complete data. This study provides a proof of concept that cTI can identify data-driven disease stages in a heterogeneous sample combining different mutations and disease stages of genetic FTD using only MRI metrics.© 2021 The Authors. Human Brain Mapping published by Wiley Periodicals LLC