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Research in cognitive science has highlighted the effectiveness of several learning techniques, and a number of studies have analyzed their prevalence among university students and their relationship with academic achievement. In this study, we surveyed a large, heterogeneous sample of secondary school students to reveal how often they use research-supported techniques in comparison with other frequent techniques, and we analyzed the association between their study strategies and school achievement. We also assessed the associations between study techniques and several students' beliefs and attitudes toward learning (self-efficacy, goal orientation, control beliefs, growth mindset, and examination anxiety). Results showed that, except for distributed practice, only those techniques that are supported by previous research yielded an association with achievement, and they exhibited higher associations with self-efficacy, growth mindset, control beliefs, and learning goal orientation than non-supported techniques. [ABSTRACT FROM AUTHOR]

Fatty acid elongase ELOVL5 is part of a protein family of multipass transmembrane proteins that reside in the endoplasmic reticulum where they regulate long-chain fatty acid elongation. A missense variant (c.689G>T p.Gly230Val) in ELOVL5 causes Spinocerebellar Ataxia subtype 38 (SCA38), a neurodegenerative disorder characterized by autosomal dominant inheritance, cerebellar Purkinje cell demise and adult-onset ataxia. Having previously showed aberrant accumulation of p.G230V in the Golgi complex, here we further investigated the pathogenic mechanisms triggered by p.G230V, integrating functional studies with bioinformatic analyses of protein sequence and structure. Biochemical analysis showed that p.G230V enzymatic activity was normal. In contrast, SCA38-derived fibroblasts showed reduced expression of ELOVL5, Golgi complex enlargement and increased proteasomal degradation with respect to controls. By heterologous overexpression, p.G230V was significantly more active than wild-type ELOVL5 in triggering the unfolded protein response and in decreasing viability in mouse cortical neurons. By homology modelling, we generated native and p.G230V protein structures whose superposition revealed a shift in Loop 6 in p.G230V that altered a highly conserved intramolecular disulphide bond. The conformation of this bond, connecting Loop 2 and Loop 6, appears to be elongase-specific. Alteration of this intramolecular interaction was also observed when comparing wild-type ELOVL4 and the p.W246G variant which causes SCA34. We demonstrate by sequence and structure analyses that ELOVL5 p.G230V and ELOVL4 p.W246G are position-equivalent missense variants. We conclude that SCA38 is a conformational disease and propose combined loss of function by mislocalization and gain of toxic function by ER/Golgi stress as early events in SCA38 pathogenesis. [ABSTRACT FROM AUTHOR]

Short term treatment with low doses of glucocorticoid analogues has been shown to ameliorate neurological symptoms in Ataxia–Telangiectasia (A–T), a rare autosomal recessive multisystem disease that mainly affects the cerebellum, immune system, and lungs. Molecular mechanisms underlying this clinical observation are unclear. We aimed at evaluating the effect of dexamethasone on the induction of alternative ATM transcripts (ATMdexa1). We showed that dexamethasone cannot induce an alternative ATM transcript in control and A–T lymphoblasts and primary fibroblasts, or in an ATM-knockout HeLa cell line. We also demonstrated that some of the reported readouts associated with ATMdexa1 are due to cellular artifacts and the direct induction of γH2AX by dexamethasone via DNA-PK. Finally, we suggest caution in interpreting dexamethasone effects in vitro for the results to be translated into a rational use of the drug in A–T patients. [ABSTRACT FROM AUTHOR]

The LEXIN database offers psycholinguistic indexes of the 13,184 different words (types) computed from 178,839 occurrences of these words (tokens) contained in a corpus of 134 beginning readers widely used in Spain. This database provides four statistical indicators: F (overall word frequency), D (index of dispersion across selected readers), U (estimated frequency per million words), and SFI (standard frequency index). It also gives information about the number of letters, syntactic category, and syllabic structure of the words included. To facilitate comparisons, LEXIN provides data from LEXESP's (Sebastiá-Gallés, Marti, Cuetos, & Carreiras, 2000), Alameda and Cuetos's (1995), and Martlnez and Garcia's (2004) Spanish adult psycholinguistic frequency databases. Access to the LEXIN database is facilitated by a computer program. The LEXIN program allows for the creation of word lists by letting the user specify searching criteria. LEXIN can be useful for researchers in cognitive psychology, particularly in the areas of psycholinguistics and education. [ABSTRACT FROM AUTHOR]