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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
Dynamic global vegetation models (DGVMs) are designed for the study of past, present and future vegetation patterns together with associated biogeochemical cycles and climate feedbacks. However, most DGVMs do not yet have detailed representations of permafrost and non-permafrost peatlands, which are an important store of carbon, particularly at high latitudes. We demonstrate a new implementation o
Estimating glomerular filtration rate (GFR) in adults by using the average of values obtained by a cystatin C- (eGFRcystatin C) and a creatinine-based (eGFRcreatinine) equation shows at least the same diagnostic performance as GFR estimates obtained by equations using only one of these analytes or by complex equations using both analytes. Comparison of eGFRcystatin C and eGFRcreatinine plays a piv
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
The present study aims at exploring the influence of voice quality on listening effort in children performing a language comprehension test with sentences of increasing difficulty. Listening effort is explored in relation to gender ( = cisgender). The study has a between-groups design. Ninety-three mainstreamed children aged 8;2 to 9;3 with typical language development participated. The children w
Background: Aβ pathology is associated with longitudinal changes of brain metabolism, atrophy, and cognition, in cognitively healthy elders. However, Aβ information is usually measured cross-sectionally and dichotomized to classify subjects as Aβ-positive or Aβ-negative, making it difficult to evaluate when brain and cognitive changes occur with respect to emerging Aβ pathology. In this study, we
Enterprise Social Media (ESM) platforms have been gaining popularity in organizations in recent years. Despite the perceived value of these platforms, many ESM projects fail in their first six months because of the lack of employee engagement. Reputation systems, which track user behaviors within the ESM and assign the user a reputation score based upon a points system designated by the organizati
This article discusses eleven Southeast Asian students' transnational mobility in order to obtain higher education at an Islamic university in Jakarta. A life story approach has been used and semi-structured and interviewee-oriented interviews have been carried out in the field, as well as on the internet. The focus is not only on the students' individual experiences, such as educational backgroun
One major concern that organizations face when deploying Enterprise Social Media (ESM) platforms is that of how to encourage employees' engagement within the system. Reputation systems that gamify the adoption process are incorporated into many ESM to inculcate employee engagement with the ESM. This study seeks to understand the dynamic between employees' online and offline performance by examinin
This study investigates how accessing user-generated content in online user innovation communities (OUICs) may influence employee creativity. By analyzing a longitudinal dataset obtained from the IdeaExchange community of Salesforce.com, we find that employees who frequently access diverse and well-codified idea content contributed by external product users are likely to generate more ideas than t
Internal crowdsourcing platforms, which enable firms to generate a large pool of ideas and connect employees to large number of colleagues, hold the potential to support employee innovation. Yet, managers face considerable difficulties when it comes to deploying such platforms in a way that generates business value. This article describes the eight years-long evolution of Allianz UK efforts to tap
In order to reduce nitrogen oxides (NOx) and soot emissions while maintaining high thermal efficiency, more advanced combustion concepts have been developed over the years, such as Homogeneous Charge Compression Ignition (HCCI) and Partially Premixed Combustion (PPC), as possible combustion processes in commercial engines. Compared to HCCI, PPC has advantages of lower unburned hydrocarbon (UHC) an
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
Increased research is being driven by the automotive industry facing challenges, requiring to comply with both current and future emissions legislation, and to lower the fuel consumption. The reason for this legislation is to restrict the harmful pollution which every year causes 3.3 million premature deaths worldwide [1]. One factor that causes this pollution is NOx emissions. NOx emission legisl
The extensive discussions of the relationship between the history of science and the philosophy of science in the mid-20th century provide a long history of grappling with the relevance of empirical research on the practices of science to the philosophical analysis of science. Further, those discussions also touched upon the issue of importing empirical methods into the philosophy of science throu
