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Proteomics insights into infantile neuronal ceroid lipofuscinosis (CLN1) point to the involvement of cilia pathology in the disease

Mutations in the depalmitoylation enzyme, palmitoyl protein thioesterase (PPT1), result in the early onset neurodegenerative disease known as Infantile Neuronal Ceroid Lipofuscinosis. Here, we provide proteomic evidence suggesting that PPT1 deficiency could be considered as a ciliopathy. Analysis of membrane proteins from brain enriched for acylated proteins from neonate Ppt1 knock out and control

Equilibrium oxygen storage capacity of ultrathin CeO2-δ depends non-monotonically on large biaxial strain

Elastic strain is being increasingly employed to enhance the catalytic properties of mixed ion-electron conducting oxides. However, its effect on oxygen storage capacity is not well established. Here, we fabricate ultrathin, coherently strained films of CeO2-δ between 5.6% biaxial compression and 2.1% tension. In situ ambient pressure X-ray photoelectron spectroscopy reveals up to a fourfold enhan

IS security menace : When security creates insecurity

Modern organizations face significant information security violations from inside the organizations to which they respond with various managerial techniques. It is widely believed in IS security literature that enforcing IS security policy compliance on employees through various means is the solution for security effectiveness. Nevertheless, this manuscript challenges that notion and advances a st

District heating and cooling systems - Framework for Modelica-based simulation and dynamic optimization

Future district heating systems (so called 4th Generation District Heating (4GDH) systems) have to address challenges such as integration of (de)centralized renewable energy sources and storage, low system temperatures and high fluctuation of the supply temperature. This paper presents a novel framework for representing and simplifying on-grid energy systems as well as for dynamic thermo-hydraulic

Geant4-aided Quantum State Selective Decay Spectroscopy of 213Ra

Utilizing the excellent mass resolving power of SHIPTRAP and the charged-particle-g multicoincidence setup TASISpec, the decay path of the 213Ra ground state can be exclusively studied. Based on virtual experiments with Geant4 it is possible to refine the a-branching ratios of the 213Ra ground state as well as g-ray branching ratios in the 209Rn daughter. The present study provides a proof of conc

Vem var Lunds förste student?

Försök att identifiera den ”Johannes Andrea fil. Bagerús” som står som första namn i Lunds universitets äldsta inskrivningsmatrikel (1667)

Influence of grid resolution and meteorological forcing on simulated European air quality: A sensitivity study with the modeling system COSMO-MUSCAT

Model evaluation studies are essential for determining model performance as well as assessing model deficiencies, and are the focus of the Air Quality Model Evaluation International Initiative (AQMEII). The chemistry-transport model system COSMO–MUSCAT participates in this initiative. In this paper the robustness and variability of the model results against changes in the model setup are analyzed.

Criminal organizing applying the theory of partial organization to four cases of organized crime

We explore how the idea of partial organization can provide insights in the study of organized crime. Studying criminal organizing with a theoretical framework used for other social organizing phenomena can help us see the interplay between different forms of criminal collaboration under a single analytical lens, and start a discussion on whether criminal organizing is intrinsically different from