Granulin Knock Out Zebrafish Lack Frontotemporal Lobar Degeneration and Neuronal Ceroid Lipofuscinosis Pathology

Loss of function mutations in granulin (GRN) are linked to two distinct neurological disorders, frontotemporal lobar degeneration (FTLD) and neuronal ceroid lipofuscinosis (NCL). It is so far unknown how a complete loss of GRN in NCL and partial loss of GRN in FTLD can result in such distinct diseases. In zebrafish, there are two GRN homologues, Granulin A (Grna) and Granulin B (Grnb). We have generated stable Grna and Grnb loss of function zebrafish mutants by zinc finger nuclease mediated genome editing. Surprisingly, the grna and grnb single and double mutants display neither spinal motor neuron axonopathies nor a reduced number of myogenic progenitor cells as previously reported for Grna and Grnb knock down embryos. Additionally, grna-/-;grnb-/- double mutants have no obvious FTLD- and NCL-related biochemical and neuropathological phenotypes. Taken together, the Grna and Grnb single and double knock out zebrafish lack any obvious morphological, pathological and biochemical phenotypes. Loss of zebrafish Grna and Grnb might therefore either be fully compensated or only become symptomatic upon additional challenge

A Taxonomy Framework for Maritime Cybersecurity: A Demonstration Using the Automatic Identification System

The maritime transportation system is increasingly a target of cyber attacks. This paper describes a taxonomy that supports the creation of adversarial cyber models, risk mitigation, and resiliency plans as applied to the maritime industry, using the Automatic Identification System as a specific illustration of the approach. This method has already been applied to the aviation sector; retooling it for a maritime example demonstrates its broad applicability to the transportation sector, in general

A Taxonomy Framework for Maritime Cybersecurity: A Demonstration Using the Automatic Identification System

The maritime transportation system is increasingly a target of cyber attacks. This paper describes a taxonomy that supports the creation of adversarial cyber models, risk mitigation, and resiliency plans as applied to the maritime industry, using the Automatic Identification System as a specific illustration of the approach. This method has already been applied to the aviation sector; retooling it for a maritime example demonstrates its broad applicability to the transportation sector, in general

Engineering report single-shell tank farms interim measures to limit infiltration through the vadose zone

Identifies, evaluates and recommends interim measures for reducing or eliminating water sources and preferential pathways within the vadose zone of the single-shell tank farms. Features studied: surface water infiltration and leaking water lines that provide recharge moisture, and wells that could provide pathways for contaminant migration. An extensive data base, maps, recommended mitigations, and rough order of magnitude costs are included

ACTRA - A Case Study for Threat Information Sharing

This paper provides a case study for information sharing within a public/private not-for-profit partnership organization called ACTRA – Arizona Cyber Threat Response Alliance, Inc.. This initiative is comprised of public and private entities with government agencies as invited guests aligned around the goal of improved response to cyber security events. Technical, political, legal and organizational issues arise when multiple parties attempt to exchange information in a formal setting. Benefits and specific solutions developed are discussed. The study concludes with several areas for future improvement and investigation as well as recommendations for newly forming sharing groups

A Taxonomy Framework for Maritime Cybersecurity: A Demonstration Using the Automatic Identification System

The maritime transportation system is increasingly a target of cyber attacks. This paper describes a taxonomy that supports the creation of adversarial cyber models, risk mitigation, and resiliency plans as applied to the maritime industry, using the Automatic Identification System as a specific illustration of the approach. This method has already been applied to the aviation sector; retooling it for a maritime example demonstrates its broad applicability to the transportation sector, in general

A study of the selective hydroconversion of biocarboxylic acids to bioalcohols over novel indium-nickel/zeolite catalysts using octanoic acid as model reactant

Octanoic acid (OA) was hydrotreated in a flow-through reactor at 21 bar total pressure and 240-340 °C over supported metal catalysts prepared from Ni-zeolites (A, X, P) by indium modification. The Ni-zeolites were activated first in H2 flow at 21 bar and 450 °C. While a fraction of the nickel got fully reduced, the zeolite structure became partially destructed. However, some nickel cations remained unreduced, therefore a large fraction of the crystalline zeolite structure was retained. The indium modification of the reduced Ni-zeolites generated bimetallic NiIn/Ni,H-zeolite catalysts having higher stability, hydroconversion activity, octanol selectivity, and lower hydrodecarbonylation activity than the parent partially destructed Ni-zeolite and the Adkins-type catalysts, commercially applied for the conversion of fatty acids to alcohols. © 2013 Akadémiai Kiadó, Budapest, Hungary

Astrocytic gap junctional communication is reduced in amyloid-β-treated cultured astrocytes, but not in Alzheimer's disease transgenic mice

Alzheimer's disease is characterized by accumulation of amyloid deposits in brain, progressive cognitive deficits and reduced glucose utilization. Many consequences of the disease are attributed to neuronal dysfunction, but roles of astrocytes in its pathogenesis are not well understood. Astrocytes are extensively coupled via gap junctions, and abnormal trafficking of metabolites and signalling molecules within astrocytic syncytia could alter functional interactions among cells comprising the neurovascular unit. To evaluate the influence of amyloid-β on astrocyte gap junctional communication, cultured astrocytes were treated with monomerized amyloid-β1–40 (1 μmol/l) for intervals ranging from 2 h to 5 days, and the areas labelled by test compounds were determined by impaling a single astrocyte with a micropipette and diffusion of material into coupled cells. Amyloid-β-treated astrocytes had rapid, sustained 50–70% reductions in the area labelled by Lucifer Yellow, anionic Alexa Fluor® dyes and energy-related compounds, 6-NBDG (a fluorescent glucose analogue), NADH and NADPH. Amyloid-β treatment also caused a transient increase in oxidative stress. In striking contrast with these results, spreading of Lucifer Yellow within astrocytic networks in brain slices from three regions of 8.5–14-month-old control and transgenic Alzheimer's model mice was variable, labelling 10–2000 cells; there were no statistically significant differences in the number of dye-labelled cells among the groups or with age. Thus amyloid-induced dysfunction of gap junctional communication in cultured astrocytes does not reflect the maintenance of dye transfer through astrocytic syncytial networks in transgenic mice; the pathophysiology of Alzheimer's disease is not appropriately represented by the cell culture system

Decoupling of optoelectronic properties from morphological changes in sodium treated kesterite thin film solar cells

Sodium is typically used during the synthesis of kesterite thin films to enhance the performance of solar cells. As sodium tends to affect grain growth and morphology, it is difficult to analyse solely the electronic effects of sodium as dopant. To decouple the structural and electronic effects from each other, two processes were designed in this work to successfully incorporate sodium into a vacuum-processed Cu2ZnSnSe4absorber without changing the morphology. A thin layer of NaF is deposited before precursor deposition (Pre-NaF) or after absorber synthesis to undergo a post deposition treatment (NaF-PDT). While composition and distribution of matrix elements remain unchanged, the sodium concentration is increased upon sodium treatment up to 140 ppm as measured by inductively coupled plasma mass spectrometry. X-ray photoelectron spectroscopy showed that the surface composition was not altered. Within its detection limit, sodium was not present at the absorber surface. For a Pre-NaF sample measured with atom probe tomography a sodium concentration of 30 ppm was measured in a grain, suggesting that sodium might segregate at grain boundaries. The additional sodium content in the film leads to an increased acceptor concentration, which results in improved open-circuit voltage and fill factor.Financial support from the Swiss National Science Foundation (SNF) in the network of the Indo-Swiss Joint Research Programme (ISJRP) [IZLIZ2_157140/1] is gratefully acknowledged. T. Schwarz is grateful for the support of the German Research Foundation (DFG) [Contract GA 2450/1-1]. R. Caballero acknowledges financial support from Spanish MINECO within the Ramón y Cajal program [RYC-2011-08521], MINECO project WINCOST [ENE2016-80788-C5-2-R] and from Spanish Ministry of Education, Culture and Sport within the José Castillejo program [CAS 15/00070

Досвід створення та функціонування Державної системи правової інформації Республіки Білорусь

Щодо досвіду створення та особливостей функціонування білоруської моделі державної системи правової інформації.Относительно опыта создания и особенностей функционирования белорусской модели государственной системы правовой информации.In relation to the experience of foundation and Рeculiarities of the Belorussia model state system of the legal information functioning