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BACKGROUND: Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterised by progressive neurological degeneration, where the rate of neurological disease progression varies depending on age at neurological onset. We report longitudinal data on functional disease progression and safety observations in patients in the international NPC Registry who received continuous treatment w

Defensive coping (DefS), oxidative stress, inflammation, and related amino acids (phenylalanine [Phe] and tyrosine [Tyr]) have been implicated in cardiovascular disease. This study assessed whether inflammation, oxidative stress, changes in essential amino acids, and altered coping strategies are correlated with subclinical vascular changes in African (n = 82) and Caucasian (n = 100) men from the

Results of a search for new phenomena in events with an energetic photon and large missing transverse momentum with the ATLAS experiment at the Large Hadron Collider are reported. The data were collected in proton-proton collisions at a centre-of-mass energy of 13 TeV and correspond to an integrated luminosity of 3.2 fb−1. The observed data are in agreement with the Standard Model expectations. Ex

A search is presented for dark matter produced in association with a hadronically decaying W or Z boson using 3.2 fb-1 of pp collisions at √ s = 13 TeV recorded by the ATLAS detector at the Large Hadron Collider. Events with a hadronic jet compatible with a W or Z boson and with large missing transverse momentum are analysed. The data are consistent with the Standard Model predictions and are inte

We do mean-field perturbation theory for U(1) lattice gauge theory in the axial gauge, and evaluate corrections from fluctuations up to fourth order for the free energy and plaquette energy. Comparing with similar results previously obtained in the Feynman gauge we find, to those orders studied, a gauge dependence of the size of the first correction term neglected with one exception. This gauge de

An expanded record (~14 m)of the Paleocene–Eocene Thermal Maximum (PETM), a transient period of extreme global warming that occurred ~56 million years ago, has been found based on calcareous nannofossil and foraminifera stratigraphy in the deep marine Río Gor section, Subbetic Zone, SE Spain. During the early Palaeogene the Subbetic Zone wa

Background: Total tau (tTau) and phosphorylated 181P tau (pTau) are supportive diagnostic cerebrospinal fluid (CSF) biomarkers for Alzheimer's disease. Manual CSF tau assays are limited by lot-to-lot and between-laboratory variability and long incubation/turnaround times. Elecsys® Total-Tau CSF and Phospho-Tau (181P) CSF immunoassays were developed for fully automated cobas e analyzers, allowing b

Background: The exact pathophysiological mechanism(s) underlying endolymphatic hydrops (EH) remain elusive. We have previously shown that chronic administration of vasopressin and inhibitors of the cAMP/cGMP degrading enzymes (PDE3, PDE4, PDE5) results in the development of EH to mice. Aims/objectives: Evaluate the ability of spironolactone, an aldosterone antagonist, to prevent EH, when induced b

Evidence of air pollution exposure, namely, ambient particulate matter (PM), during pregnancy and an increased risk of autism in children is growing; however, the unique PM sources that contribute to this association are currently unknown. The aim of the present study was to investigate local, source-specific ambient PM exposure during pregnancy and its associations with childhood autism, specific

The association between pesticide exposure and non-Hodgkin lymphoma (NHL) including hairy cell leukemia (HCL) was analyzed in a pooled study of three case-control studies. Results on exposure to pesticides were based on 1,425 cases and 2,157 controls participating in the studies. Exposures were assessed by self-administered questionnaires completed as needed by phone. In the pooled univariate anal

A search for invisible decays of the Higgs boson as well as searches for dark matter candidates, produced together with a leptonically decaying Z boson, are presented. The analysis is performed using proton−proton collisions at a centre-of-mass energy of 13 TeV, delivered by the LHC, corresponding to an integrated luminosity of 139 fb−1 and recorded by the ATLAS experiment. Assuming Standard Model

With rapid urbanization and an increasing share of anthropogenic greenhouse gas emissions from cities, low-carbon urban transformations are critical for mitigating climate change. Local climate networks have been recognized for their potential to govern such activities. However, less is known about how such networks are implemented and managed as well as their capacity to accelerate transformative

Cambodian migrants in the Thai construction sector, work in precarious working environments, with limited safety conditions and low wages. The use of social protection schemes has increased in recent decades, as a method of reducing the vulnerable against poverty and livelihood risks. However, despite the transformative potential of social protection, migrant workers face difficulties in accessing

Corporations and in particular large multinational enterprises are important for every countries economic growth. However, exploitation and human rights abuses due to corporate activity are repeatedly occurring in states that lacks the proper legislation, enforcement or institutions to ensure human rights protection. Thus, there is an increasing call for lead firms of MNEs to exercise human rights

The International Criminal Court (ICC) was created as a compliment to domestic courts in the global fight against impunity. However, customary international law has afforded Heads of State with immunity from prosecution, even for serious international crimes. Some key challenges of international criminal law are to reconcile the competing objectives of maintaining stable international relations, a