n in patients with TBPI after contralateral C7 transfer.Mutations in OPTN are associated with glaucoma, an eye disease, and also with amyotrophic lateral sclerosis (ALS), a motor neuron disease. A 2-bp insertion in OPTN (691_692insAG or 2bpIns-OPTN) is associated with both glaucoma and ALS. This mutation results in frame shift after 127 amino acids, giving rise to a protein with C-terminal aberrant sequence. We have explored the mechanism of induction of cell death by this mutant in a motor neuron cell line, NSC-34, and also in a retinal cell line, 661W. Compared to wild-type OPTN, this mutant induced more cell death in NSC-34 and 661W cells. This mutant localizes predominantly in the nucleus whereas normal OPTN localizes in the cytoplasm. Deletion analysis of 2bpIns-OPTN showed that the aberrant sequence was not essential for cell death induction. This mutant interacts with TANK-binding kinase 1 (Tbk1) but not with OPTN and activates Tbk1. This mutant induced ER stress in NSC-34 cells as seen by induction of C/EBP homologous protein (CHOP) and some other genes. Induction of CHOP, autophagosomal protein LC3-II and cell death by this mutant were abrogated by Tbk1 knockdown and also by 4-phenylbutyric acid, that inhibits ER stress. Induction of CHOP and cell death by 2bpIns-OPTN was autophagy dependent as shown by the effect of Atg5 knockdown. This mutant caused increased formation of LC3-positive aggregates. Treatment of cells with autophagy inducer rapamycin reduced LC3-positive aggregates, CHOP and cell death induced by 2bpIns-OPTN. These results suggest that constitutive activation of Tbk1 by 2bpIns-OPTN leads to impaired autophagy that results in ER stress and cell death.The propagation of conformational strains by templated seeding is central to the prion concept. Seeded assembly of α-synuclein into filaments is believed to underlie the prion-like spreading of protein inclusions in a number of human neurodegenerative diseases, including Parkinson's disease, dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). We previously determined the atomic structures of α-synuclein filaments from the putamen of five individuals with MSA. Here, we used filament preparations from three of these brains for the in vitro seeded assembly of recombinant human α-synuclein. We find that the structures of the seeded assemblies differ from those of the seeds, suggesting that additional, as yet unknown, factors play a role in the propagation of the seeds. Identification of these factors will be essential for understanding the prion-like spreading of α-synuclein proteinopathies.Amniote tails display a wide variety of features for adaptation to diverse environments. Each feature originates from its own distinct developmental processes, and these processes in turn attest to an organism's evolutionary history. In this perspective, we discuss the ontogeny of tails from embryonic to adult stages, amniote tail regeneration, the mechanisms underlying tail length and neural systems, and the benefits of studying tails across vertebrates, in mammals, birds, and non-avian reptiles. The aim of this study was to provide a reliable instrument to assess evidence-based practice (EBP) dimensions in French-speaking dental students. To do so, a transcultural adaptation into French of the self-administered KACE questionnaire (Knowledge, Attitude, Access and Confidence Evaluation) and the evaluation of its psychometric properties in a sample of French dental students and teachers were performed. The effects of individual and academic factors on student performance in the KACE were also investigated. Teachers and fourth-year students completed once the translated KACE and fifth-year students completed twice the questionnaire before and after an epidemiology course. Cronbach's alpha coefficient analysis, Kruskal-Wallis and post hoc Dunn's tests, paired samples Wilcoxon tests and a generalised linear mixed model were performed to assess the psychometric properties (reliability, internal consistency, discriminant validity and responsiveness) of the questionnaire and to identify the factors associated with KACE scores, respectively. 186 participants completed the survey. Cronbach's alpha ranged from 0.06 to 0.48 for Knowledge, 0.21 to 0.59 for Attitude, 0.42 to 0.76 for Access and 0.82 to 0.93 for Confidence. The discriminant value and sensitivity of the French KACE revealed significant differences amongst students and teachers and accurately identified the effect the training course had on the students who had completed it. Preparing for a residency programme or having received add-on modules in science improved the students' performance in the test. Being female negatively impacted the KACE Confidence score. This version of the KACE is appropriate to assess EBP dimensions in French dental students. Academic background and gender may affect test performance.This version of the KACE is appropriate to assess EBP dimensions in French dental students. Academic background and gender may affect test performance. To determine the contemporary educational experiences of predoctoral dental students in the United States and Canada regarding behaviour guidance (BG) of the child patient and assess trends from a previous study in 2004. Data were collected from 32 predoctoral paediatric dentistry programme directors in the United States and Canada via a web-based survey. The didactic curriculum hours devoted to the teaching of BG techniques in 2019 are similar to 2004. A majority (60.7%) of programmes do not have a formal assessment of competency with BG techniques. Lectures (n=28), clinical experience (n=28) and observation (n=26) were the most common techniques implemented to teach BG techniques, and tell-show-do (100%), non-verbal communication (82.1%), positive reinforcement (89.3%) and distraction (82.1%) were the techniques that more than 75% of dental students most commonly have hands-on experience with during their dental education. learn more In 2019, students tended to have more hands-on experience with nitrous oxide/oxygen inhalation and less hands-on experience with aversive techniques and sedation. The majority of dental schools do not have a formal competency in BG of the child patient. Compared with 2004, nitrous oxide/oxygen is used more by dental students and there is less predoctoral education in aversive BG techniques.The majority of dental schools do not have a formal competency in BG of the child patient. Compared with 2004, nitrous oxide/oxygen is used more by dental students and there is less predoctoral education in aversive BG techniques.