Mutations in the SPATA5 gene are associated with epilepsy, hearing loss and mental retardation syndrome (EHLMRS). While SPATA5 is ubiquitously expressed and is attributed a role within mitochondrial morphogenesis during spermatogenesis, there is only limited knowledge about the associated muscular and molecular pathology. This study reports on a comprehensive workup of muscular pathology, including proteomic profiling and microscopic studies, performed on an 8-year-old girl with typical clinical presentation of EHLMRS, where exome analysis revealed two clinically relevant, compound-heterozygous variants in SPATA5. Proteomic profiling of a quadriceps biopsy showed the dysregulation of 82 proteins, out of which 15 were localized in the mitochondrion, while 19 were associated with diseases presenting with phenotypical overlap to EHLMRS. Histological staining of our patient’s muscle biopsy hints towards mitochondrial pathology, while the identification of dysregulated proteins attested to the vulnerability of the cell beyond the mitochondria. Through our study we provide insights into the molecular etiology of EHLMRS and provide further evidence for a muscle pathology associated with SPATA5 deficiency, including a pathological histochemical pattern accompanied by dysregulated protein expression. 相似文献
A two-bed hospital ward with one standing healthcare person and a ceiling-mounted lowimpulse semicircular inlet diffuser is simulated in a full-scale room. Tracer gas is used for simulating gaseous contaminants, and the concentration is measured at different air change rates and different postures of the patients. A textile partition between the beds, which is typical in a hospital ward, is used for protection of the patients in some of the experiments. Three different layouts of return openings are tested. One layout with one opening at the ceiling, another with four openings at the wall opposite to the inlet diffuser, and one with a high location of these four openings. The downward recirculating flow is on average parallel with the partition, and in most cases the partition does not decrease cross-infection. A high location of the four return openings decreases the risk of cross-infection. 相似文献
For decades, motorsport has been an incubator for innovations in the automotive sector and brought forth systems, like, disk brakes or rearview mirrors. Autonomous racing series such as Roborace, F1Tenth, or the Indy Autonomous Challenge (IAC) are envisioned as playing a similar role within the autonomous vehicle sector, serving as a proving ground for new technology at the limits of the autonomous systems capabilities. This paper outlines the software stack and approach of the TUM Autonomous Motorsport team for their participation in the IAC, which holds two competitions: A single-vehicle competition on the Indianapolis Motor Speedway and a passing competition at the Las Vegas Motor Speedway. Nine university teams used an identical vehicle platform: A modified Indy Lights chassis equipped with sensors, a computing platform, and actuators. All the teams developed different algorithms for object detection, localization, planning, prediction, and control of the race cars. The team from Technical University of Munich (TUM) placed first in Indianapolis and secured second place in Las Vegas. During the final of the passing competition, the TUM team reached speeds and accelerations close to the limit of the vehicle, peaking at around and . This paper will present details of the vehicle hardware platform, the developed algorithms, and the workflow to test and enhance the software applied during the 2-year project. We derive deep insights into the autonomous vehicle's behavior at high speed and high acceleration by providing a detailed competition analysis. On the basis of this, we deduce a list of lessons learned and provide insights on promising areas of future work based on the real-world evaluation of the displayed concepts. 相似文献
The shells of walnuts (WS) are major refuse in the global fruits and nuts trade. This, otherwise discarded, lignin-rich material can be carbonized to biochar—a value-added product with environmental applications such as carbon sequestration, soil amelioration, and pollutant adsorption. These applications are dictated by structural and chemical characteristics of the biochar carbon. Conventional controlled pyrolysis (CPy) of biomass is cost-intensive and technically too complex for widespread adoption, especially in emerging economies. Here, walnut shell biochar (BWS0) is derived through uncontrolled pyrolysis (UCPy) in a pyrolysis oven and further hybridized as magnetic biochar through ex-situ chemical co-precipitation. The physico-chemical characteristics of biochar and its water-extractable fractions are comprehensively investigated to understand their carbon structure and environmental applicability. The sp2 amorphous carbon sequestered in BWS0 is 0.84 kgCO2/kgbiomass with a BET (N2) surface area of 292 m2/g and is comparable to biochar from CPy in terms of carbon structure. The polyaromatic hydrocarbons present are only trace amounts of naphthalene, biphenyl, and phenanthrene. The magnetization decreases porosity of BWS0 while greatly facilitating its separation from aqueous media. BWS0 is suitable for adsorption of cations (between pH 2.8 and 9.45) and hydrophobic pollutants with only 19 mg L−1 fouling from their intrinsic dissolved organic carbon. In combination with fast-release N, P fertilizers, BWS0 (C/N of 24.8) is suitable for application in hydrophilic soils at higher loading rates. The results suggest an avenue where WS biochar can also be prepared via UCPy for direct environmental applications. Future investigations into soil incubation and adsorption tests are recommended.