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The nuclear pore complex (NPC) is among the most elaborate protein complexes in eukaryotes. While ribosomes and proteasomes are known to require dedicated assembly machinery, our understanding of NPC assembly is at a relatively early stage. Defects in NPC assembly or homeostasis are tied to movement disorders, including dystonia and amyotrophic lateral sclerosis (ALS), as well as aging, requiring a better understanding of these processes to enable therapeutic intervention. Here, we discuss recent progress in the understanding of NPC assembly and highlight how related defects in human disorders can shed light on NPC biogenesis. We propose that the condensation of phenylalanine‐glycine repeat nucleoporins needs to be carefully controlled during NPC assembly to prevent aberrant condensation, aggregation, or amyloid formation. [ABSTRACT FROM AUTHOR]

Background Pruritus is a common symptom associated with cholestatic liver diseases. To date only small single centre case series have suggested efficacy of nasobiliary drainage in relieving cholestatic pruritus. Aim To perform a multicentre study to evaluate the safety and efficacy of nasobiliary drainage in cholestatic pruritus. Methods This was a retrospective study of all patients treated with nasobiliary drainage for refractory cholestatic pruritus between 2006 and 2015 at five European centres. Pruritus was quantified using a visual analogue scale (VAS) and liver enzymes, serum bilirubin and total serum bile salts (TBS) were measured before (pre-NBD) and after nasobiliary drainage (post-NBD). We analysed the duration of treatment response and associated complications. Results In total, 27 patients (59% females) underwent 29 nasobiliary drainage procedures. The median duration of NBD was 7 days. NBD decreased pruritus in 89.6% of cases (VAS from 10.0 to 0.3, P < 0.0001). The median percentage decline in pruritus VAS was 94% and 33% of patients were free of pruritus within 24 h of starting drainage. The duration of treatment response was independent of duration of drainage (P = 0.12) and bile output. Significant improvements were seen in the median levels of serum alkaline phosphatase (P = 0.001) and serum bilirubin (P = 0.03) but not in serum TBS (P = 0.07). Mild post-endoscopic retrograde cholangiopancreatography pancreatitis (31%) was the most frequent complication. Conclusions Nasobiliary drainage is effective in relieving cholestatic pruritus in most patients and has favourable effect on biomarkers of cholestasis. Nasobiliary drainage may be associated with high risk of adverse events, especially pancreatitis. Prospective studies are needed to confirm our findings. [ABSTRACT FROM AUTHOR]

This study reports on the effects of the combined use of an interactive whiteboard ( IWB) and a virtual learning environment ( VLE) on mathematics performance and motivation. Lessons taught with an IWB were made available on the VLE, so that they could be consulted regardless of time and place. Students' mathematics performance was monitored during one school year and their motivation for mathematics was followed for 3 years. There was no relation between frequency of being taught with an IWB and mathematics performance. However, students' motivation for mathematics appeared to be positively related to the combination of lessons made for the IWB and availability of these lessons on the VLE. This availability was also the main reason for students' appreciation of the VLE. Students appeared to use the VLE mostly to prepare for tests and examinations. [ABSTRACT FROM AUTHOR]

The fit between existing educational practices and promoted classroom use of information and communication technologies is increasingly recognized as a factor in successful integration of such technologies in classroom practice. Using a descriptive multiple-case study design, we characterize the types of information and communication technology (ICT) use resulting from a 'concept-guided' approach in five schools. Teachers at schools characterized as having a 'traditional' or an 'innovative' school concept were supported in designing and implementing ICT-enhanced learning arrangements that fit their schools' educational concept. In the traditional schools, ICT was primarily applied to extend and support the use of standard teaching materials in mostly teacher-directed activities. The innovative schools on the other hand used ICT tools primarily to support open-ended activities with a lot of input from the pupils. All five schools expected ICT to increase pupils' motivation, improve learning results, promote self-directed learning, and enable differentiation between pupils. Yet underneath these goals, we find distinct differences in expectations between the two school types. [ABSTRACT FROM AUTHOR]

The serum immunoglobulin levels of 200 first-degree relatives and 23 spouses of 32 patients with myelomatosis were compared with those of age- and sexmatched control persons. First-degree relatives of myeloma patients appeared to have higher serum levels of IgG (P < 0.05), IgA (P < 0.05), and IgM (P < . 0.01) than their age- and sex-matched controls. The spouses as a group did not differ from their controls in this respect. No statistically significant difference in the incidence of M-components among family members (0.6%) and spouses (4.3%) was found compared with the incidence in the general population (0.9%). Myelomatosis as such appeared to be more frequent than in the general population (P < 0.05). [ABSTRACT FROM AUTHOR]