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- Farber disease (3)
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The application of electrical resistivity tomography to peatlands supports conventional coring by providing data on the current condition of peatlands, including data on stratigraphy, peat properties and thickness of organic deposits. Data on the current condition of drained peatlands are particularly required to improve estimates of carbon storage as well as losses and emissions from agriculturally used peatlands. However, most of the studies focusing on electrical resistivity tomography surveys have been conducted on natural peatlands with higher groundwater levels. Peatlands drained for agriculture have not often been studied using geophysical techniques. Drained sites are characterized by low groundwater levels and high groundwater fluctuations during the year, which lead to varying levels of water saturation. To validate better electrical resistivity tomography surveys of drained peatlands, the aim of this laboratory study is to investigate the influence of varying water saturation levels on electrical conductivity (reciprocal of resistivity) for a variety of peat and gyttja types, as well as for different degrees of peat decomposition. Results show that different levels of water saturation strongly influence bulk electrical conductivity. Distinct differences in this relationship exist between peat and gyttja substrates and between different degrees of peat decomposition. Peat shows an exponential relationship for all degrees of decomposition, whereas gyttja, in particular organic-rich gyttja, is characterized by a rather unimodal relationship. The slopes for the relationship between electrical conductivity and water content are steeper at high degrees of decomposition than for peat of low degrees of decomposition. These results have direct implications for field electrical resistivity tomography surveys. In drained peatlands that are strongly susceptible to drying, electrical resistivity tomography surveys have a high potential to monitor the actual field water content. In addition, at comparable water saturations, high or low degrees of decomposition can be inferred from electrical conductivity.
The determination of the total carbon storage of peatlands is of high relevance in the context of climate-change mitigation efforts. This determination relies on data about stratigraphy and peat properties, which are conventionally collected by coring. Ground-penetrating radar (GPR) and electrical resistivity imaging (ERI) can support these point data by providing subsoil information in two-dimensional cross-sections. In this study, GPR and ERI were conducted at two groundwater-fed fen sites located in the temperate zone in north-east Germany. The fens of this region are embedded in low conductive glacial sand and are characterised by thick layers of gyttja, which can be either mineral or organic. The two study sites are representative of this region with respect to stratigraphy (total thickness, peat and gyttja types) and ecological conditions (pH-value, trophic condition). The aim of this study is to assess the suitability of GPR and ERI to detect stratigraphy and peat properties under these characteristic site conditions. Results show that GPR clearly detects the interfaces between (i) Carex and brown-moss peat, (ii) brown-moss peat and organic gyttja, (iii) organic- and mineral gyttja, and (iv) mineral gyttja and the parent material (glacial sand). These layers differ in bulk density and the related organic matter content. ERI, however, does not delineate these layers; rather it delineates regions of varying properties. At our base-rich site, pore fluid conductivity and cation.exchange capacity are the main factors that determine peat electrical conductivity (reverse of resistivity), whereas organic matter and water content are most influential at the more acidic site. Thus the correlation between peat properties and electrical conductivity are driven by site-specific conditions, which are mainly determined by the solute load in the groundwater at fens. When the total organic deposits exceed a thickness of 5 m, the depth of investigation by GPR is limited due to increasing attenuation. This is not a limiting factor for ERI, where the transition from organic deposits to glacial sand is visible at both sites. Due to these specific sensitivities, a combined application of GPR and ERI meets the demand for up-to-date information on carbon storage of peatlands, which is, moreover, very site-specific because of the inherent variety of ecological conditions and stratigraphy between peatlands in general and between fens and bogs in particular. (C) 2016 Elsevier B.V. All rights reserved.
High nitrogen (N) efficiency, characterized by high grain yield under N limitation, is an important agricultural trait in Brassica napus L. cultivars related to delayed senescence of older leaves during reproductive growth (a syndrome called stay-green). The aim of this study was thus to identify genes whose expression is specifically altered during N starvation-induced leaf senescence and that can be used as markers to distinguish cultivars at early stages of senescence prior to chlorophyll loss. To this end, the transcriptomes of leaves of two B. napus cultivars differing in stay-green characteristics and N efficiency were analyzed 4 days after the induction of senescence by either N starvation, leaf shading or detaching. In addition to N metabolism genes, N starvation mostly (and specifically) repressed genes related to photosynthesis, photorespiration and cell-wall structure, while genes related to mitochondrial electron transport and flavonoid biosynthesis were predominately up-regulated. A kinetic study over a period of 12 days with four B. napus cultivars differing in their stay-green characteristics confirmed the cultivar-specific regulation of six genes in agreement with their senescence behavior: the senescence regulator ANAC029, the anthocyanin synthesis-related genes ANS and DFR-like1, the ammonium transporter AMT1:4, the ureide transporter UPSS, and SPS1 involved in sucrose biosynthesis. The identified genes represent markers for the detection of cultivar-specific differences in N starvation-induced leaf senescence and can thus be employed as valuable tools in B. napus breeding. (C) 2015 Elsevier Ireland Ltd. All rights reserved.
Farber disease is a rare lysosomal storage disorder resulting from acid ceramidase deficiency and subsequent ceramide accumulation. No treatments for Farber disease are clinically available, and affected patients have a severely shortened lifespan. We have recently reported a novel acid ceramidase deficiency model that mirrors the human disease closely. Acid sphingomyelinase is the enzyme that generates ceramide upstream of acid ceramidase in the lysosomes. Using our acid ceramidase deficiency model, we tested if acid sphingomyelinase could be a potential novel therapeutic target for the treatment of Farber disease. A number of functional acid sphingomyelinase inhibitors are clinically available and have been used for decades to treat major depression. Using these as a therapeutic for Farber disease, thus, has the potential to improve central nervous symptoms of the disease as well, something all other treatment options for Farber disease can’t achieve so far. As a proof-of-concept study, we first cross-bred acid ceramidase deficient mice with acid sphingomyelinase deficient mice in order to prevent ceramide accumulation. Double-deficient mice had reduced ceramide accumulation, fewer disease manifestations, and prolonged survival. We next targeted acid sphingomyelinase pharmacologically, to test if these findings would translate to a setting with clinical applicability. Surprisingly, the treatment of acid ceramidase deficient mice with the acid sphingomyelinase inhibitor amitriptyline was toxic to acid ceramidase deficient mice and killed them within a few days of treatment. In conclusion, our study provides the first proof-of-concept that acid sphingomyelinase could be a potential new therapeutic target for Farber disease to reduce disease manifestations and prolong survival. However, we also identified previously unknown toxicity of the functional acid sphingomyelinase inhibitor amitriptyline in the context of Farber disease, strongly cautioning against the use of this substance class for Farber disease patients.
Farber disease is a rare lysosomal storage disorder resulting from acid ceramidase deficiency and subsequent ceramide accumulation. No treatments for Farber disease are clinically available, and affected patients have a severely shortened lifespan. We have recently reported a novel acid ceramidase deficiency model that mirrors the human disease closely. Acid sphingomyelinase is the enzyme that generates ceramide upstream of acid ceramidase in the lysosomes. Using our acid ceramidase deficiency model, we tested if acid sphingomyelinase could be a potential novel therapeutic target for the treatment of Farber disease. A number of functional acid sphingomyelinase inhibitors are clinically available and have been used for decades to treat major depression. Using these as a therapeutic for Farber disease, thus, has the potential to improve central nervous symptoms of the disease as well, something all other treatment options for Farber disease can’t achieve so far. As a proof-of-concept study, we first cross-bred acid ceramidase deficient mice with acid sphingomyelinase deficient mice in order to prevent ceramide accumulation. Double-deficient mice had reduced ceramide accumulation, fewer disease manifestations, and prolonged survival. We next targeted acid sphingomyelinase pharmacologically, to test if these findings would translate to a setting with clinical applicability. Surprisingly, the treatment of acid ceramidase deficient mice with the acid sphingomyelinase inhibitor amitriptyline was toxic to acid ceramidase deficient mice and killed them within a few days of treatment. In conclusion, our study provides the first proof-of-concept that acid sphingomyelinase could be a potential new therapeutic target for Farber disease to reduce disease manifestations and prolong survival. However, we also identified previously unknown toxicity of the functional acid sphingomyelinase inhibitor amitriptyline in the context of Farber disease, strongly cautioning against the use of this substance class for Farber disease patients
Farber disease (FD) is a rare lysosomal storage disorder resulting from acid ceramidase deficiency and subsequent ceramide accumulation. No treatments are clinically available and affected patients have a severely shortened lifespan. Due to the low incidence, the pathogenesis of FD is still poorly understood. Here, we report a novel acid ceramidase mutant mouse model that enables the study of pathogenic mechanisms of FD and ceramide accumulation. Asah1(tmEx1) mice were generated by deletion of the acid ceramidase signal peptide sequence. The effects on lysosomal targeting and activity of the enzyme were assessed. Ceramide and sphingomyelin levels were quantified by liquid chromatography tandem-mass spectrometry (LC-MS/MS) and disease manifestations in several organ systems were analyzed by histology and biochemistry. We show that deletion of the signal peptide sequence disrupts lysosomal targeting and enzyme activity, resulting in ceramide and sphingomyelin accumulation. The affected mice fail to thrive and die early. Histiocytic infiltrations were observed in many tissues, as well as lung inflammation, liver fibrosis, muscular disease manifestations and mild kidney injury. Our new mouse model mirrors human FD and thus offers further insights into the pathogenesis of this disease. In the future, it may also facilitate the development of urgently needed therapies.