Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination

Dysfunctional mitochondria are linked to several neurodegenerative diseases. Metabolic defects, a symptom which can result from dysfunctional mitochondria, are also present in spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease, the most frequent, dominantly inherited neurodegenerative ataxia worldwide. Mitochondrial dysfunction has been reported for several neurodegenerative disorders and ataxin-3 is known to deubiquitinylate parkin, a key protein required for canonical mitophagy. In this study, we analyzed mitochondrial function and mitophagy in a patient-derived SCA3 cell model. Human fibroblast lines isolated from SCA3 patients were immortalized and characterized. SCA3 patient fibroblasts revealed circular, ring-shaped mitochondria and featured reduced OXPHOS complexes, ATP production and cell viability. We show that wildtype ataxin-3 deubiquitinates VDAC1 (voltage-dependent anion channel 1), a member of the mitochondrial permeability transition pore and a parkin substrate. In SCA3 patients, VDAC1 deubiquitination and parkin recruitment to the depolarized mitochondria is inhibited. Increased p62-linked mitophagy, autophagosome formation and autophagy is observed under disease conditions, which is in line with mitochondrial fission. SCA3 fibroblast lines demonstrated a mitochondrial phenotype and dysregulation of parkin-VDAC1-mediated mitophagy, thereby promoting mitochondrial quality control via alternative pathways.     

工作靴内底特性检测

该文研究内底革的实用性,尤其是用于工作靴的内底。因为该类鞋用于条件恶劣的环境,要求穿着舒适,利于健康。重要的两方面是:环境卫生性能和关于明确的肌肉运动体系的物理性能。人们的脚每天频繁运动,容易失水且使表面硬化,如果鞋内底能解决此问题则是既耗费低又简单的方法。对各种材料的内底进行检测发现能承受最大撞击力的材料是硅内底和胶原填充内底。     

Sonochemical Activation of Al/Ni Hydrogenation Catalyst

This paper proposes a sonochemical approach to the nanostructuring of Al/Ni catalyst with high content of accessible Ni centers and a high reusability. The surface and bulk composition as well as pore size distribution of this catalyst are controlled synergistically by adjusting the ultrasound intensity in aqueous solution. Sonochemical activation of Al/Ni alloy leads to formation of mesoporous Al/Ni metallic based frameworks with surface area up to 125 m² g^?1, and regular distribution of nickel active center in the porous matrix. One of the opportunities of porous Al/Ni catalyst is that due to a time‐resolved controllable formation of protective oxide layer it can be stored and handled under air in comparison to traditional Raney catalysts which need inert conditions. The Al/Ni catalyst is characterized by scanning electron microscopy (SEM), electron diffraction spectroscopy (EDS), X‐ray photoelectron spectroscopy (XPS), confocal scanning fluorescence microscopy (CSFM), solid‐state NMR experiments, and powder X‐ray diffraction analysis (PXRD). The catalytic activity was investigated for the hydrogenation of acetophenone.     

Determination of aryloxyphenoxypropanoic acid derivatives in crops treated with herbicidal sprays

Summary A reproducible and sensitive gas Chromatographic method has been developed for the trace analysis of fluazifop-butyl, haloxyfop-ethoxyethyl and quizalofop-ethyl as well as their main metabolites (corresponding acids analysed after conversion to methyl esters) in some fruits and vegetables treated with herbicidal sprays. Comparable detection limits (0.01 mg/kg) were achieved when mass fragmentography and/or the electron capture detector were employed. However, in the latter case, bromination of fluazifop esters had to be carried out prior to GC analysis. By means of the nitrogen-phosphorus detector, determination of residues at a level of 0.05 mg/kg was possible. The residue values found in strawberries, cabbages, cauliflowers and carrots were used to discuss the degree of metabolic hydrolysis of parent esters, i.e. active ingredients of herbicides.

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Bestimmung von Aryloxyphenoxypropionsäure-Derivaten in mit Herbicidsprays behandelten Produkten

Zusammenfassung Es wurde eine gaschromatographische Methode zur quantitativen und selektiven Bestimmung von Fluazifop-butyl, Haloxyfop-ethoxyethyl und Quizalofop-ethyl sowie von ihren Hauptmetaboliten (den entsprechenden Säuren, die zu Methylestern umgesetzt werden müssen) in mit Herbiciden behandelten Obst- und Gemüseproben entwickelt. Eine Nachweisgrenze von 0,01 mg/kg wurde durch Elektroneneinfanggaschromatographie oder durch Massenfragmentographie erzielt. Um die gleiche Empfindlichkeit des EC-Detektors bei Fluazifop zu erreichen, wurden die beiden Ester mit Brom umgesetzt. Mit Hilfe des NP-Detektors konnte eine Nachweisgrenze von 0,05 mg/kg erreicht werden. Die festgestellten Rückstandsgehalte in Erdbeeren, Kohl, Blumenkohl und Möhren wurden gleichzeitig zur Beurteilung der metabolischen Hydrolyse der diskutierten Ester (der Wirkstoffe der Herbicide) herangezogen.

     

iMobile EE – An Enterprise Mobile Service Platform

iMobile is an enterprise mobile service platform that allows resource-limited mobile devices to communicate with each other and to securely access corporate contents and services. The original iMobile architecture consists of devlets that provide protocol interfaces to different mobile devices and infolets that access and transcode information based on device profiles. iMobile Enterprise Edition (iMobile EE) is a redesign of the original iMobile architecture to address the security, scalability, and availability requirements of a large enterprise such as AT&T. iMobile EE incorporates gateways that interact with corporate authentication services, replicated iMobile servers with backend connections to corporate services, a reliable message queue that connects iMobile gateways and servers, and a comprehensive service profile database that governs operations of the mobile service platform. The iMobile EE architecture was also extended to provide personalized multimedia services, allowing mobile users to remotely control, record, and request video contents. iMobile EE aims to provide a scalable, secure, and modular software platform that makes enterprise services easily accessible to a growing list of mobile devices roaming among various wireless networks.     

Degradation studies of transparent conducting oxide: a substrate for microcrystalline silicon thin film solar cells

Aluminium doped ZnO films have been developed by RF-magnetron sputtering at 350 °C substrate temperature on glass substrate and commercially available SnO₂-coated glass substrate. The developed ZnO and SnO₂/ZnO films can be used as the substrates of microcrystalline silicon based solar cell. The electrical, optical properties and surface morphologies of ZnO film and SnO₂/ZnO bi-layer films have been investigated and they are compared with the commercially available SnO_2-coated glass substrate. The resistivities of ZnO and SnO₂ films are comparable (10⁻⁴ Ω-cm). Surface morphologies of different transparent conducting oxide coated substrates before and after H-plasma exposure were studied by scanning electron microscopy. The optical transmission of ZnO, SnO₂/ZnO and SnO₂ films are comparable and varies from 85 to 90% in the visible region. The optical transmission reduces drastically to less than 20% in SnO₂ films and for ZnO film it remains almost unchanged after H-plasma exposure. For SnO₂/ZnO film transmission decreases slightly but remains considerably high (80%). The performance of microcrystalline silicon solar cells fabricated on different transparent conducting oxides as substrates (ZnO/glass, SnO₂/glass and ZnO/SnO₂/glass double layer) is investigated in detail.     

Superradiant Emission from Coherent Excitons in van Der Waals Heterostructures

Recent advancements in isolation and stacking of layered van der Waals materials have created an unprecedented paradigm for demonstrating varieties of 2D quantum materials. Rationally designed van der Waals heterostructures composed of monolayer transition-metal dichalcogenides (TMDs) and few-layer hBN show several unique optoelectronic features driven by correlations. However, entangled superradiant excitonic species in such systems have not been observed before. In this report, it is demonstrated that strong suppression of phonon population at low temperature results in a formation of a coherent excitonic-dipoles ensemble in the heterostructure, and the collective oscillation of those dipoles stimulates a robust phase synchronized ultra-narrow band superradiant emission even at extremely low pumping intensity. Such emitters are in high demand for a multitude of applications, including fundamental research on many-body correlations and other state-of-the-art technologies. This timely demonstration paves the way for further exploration of ultralow-threshold quantum-emitting devices with unmatched design freedom and spectral tunability.     

Determination of PCBs in fatty tissues by means of several detection techniques

Difficulties with harmonization of analytical procedures and consequently poor comparability of generated data represent in the Czech Republic the main reason for the delay in issuing of updated legislation for polychlorobiphenyls (PCBs) in foodstuffs. This study draws attention to possible errors (overestimations) that can occur during routine determination of these residues in the fat portion of biotic matrices. We demonstrate the gas Chromatographic (GC) conditions under which interfering contaminants such as phthalates and/or chlorinated pesticides can be separated from analytes. Discussion is focused on the advantages and drawbacks of GC with an electron capture detector, GC mass spectrometry and GC with an atomic emission detector. Various approaches used for calculation of PCBs contents are compared.

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Bestimmung der PCB in Fettgeweben mittels verschiedener Detektionstechniken

Zusammenfassung Das Problem mit der Harmonisierung der analytischen Verfahren und der geringen Vergleichbarkeit der Angaben ist ein Hauptgrund der Verspätung in der neuen Legislative für PCB in Lebensmitteln in der Tschechischen Republik. Die Arbeit beschreibt die möglichen Fehler (Falsch-positive Fehler), die durch die routinierte Bestimmung von PCB-Rückständen in Fettanteilen der biologischen Materialien auftreten können. Es werden die gaschromatographischen Bedingungen beschrieben, unter denen die Interferenzen, wie z. B. Phthalaten und chlorierten Pesticiden, von Stoffen abgetrennt werden können. Die Vorteile und Nachteile der Methoden GC-ECD, GC-MS und GC-AED werden diskutiert. Die verschiedenen für die Kalkulation des PCB-Gehaltes angewendeten Annäherungen werden miteinander verglichen.