THE SPINOCEREBELLAR ataxias (SCAs) are neurodegenerative disorders that are clinically and genetically heterogeneous. Ten genetically different SCAs are known to be caused by trinucleotide repeat (TNR) expansions. In the dominant SCAs, the mutant proteins show an expanded polyglutamine tract in SCA1, SCA2, Machado-Joseph disease (MJD), SCA6, SCA7, and dentatorubropallidoluysian atrophy (DRPLA),^1-9 whereas SCA8 and SCA12 are caused by untranslated (CTG)_n and (CAG)_n expansions, respectively.^10,11 In Friedreich ataxia (FRDA), the mutant protein is deficient in homozygotes for a (GAA)_n expansion in intron 1 of the FRDA gene.¹² Recently, an expanded CAG repeat tract has been found at the TATA-binding protein gene (TBP) in an isolated patient with symptoms of ataxia and intellectual deterioration.¹³TBP repeat expansions have since been described in 4 Japanese families affected by a new type of ataxia with dementia named SCA17.¹⁴ Matsuura et al¹⁵ found a large expansion of pentanucleotide (ATTCT)_n in intron 9 of the SCA10 gene in patients with spinocerebellar ataxia type 10, thus being the first to show the existence of a new class of dynamic mutations.

Motivation theory is indispensable when discussing processes of learning. Learners who are motivated can learn almost everything. Students’ motivation is probably one of the most important factors for teacher effectiveness both for engagement in the learning process and high academic performance. To have effective environmental education, it is not only necessary to inform the public about the ocean but also to involve and engage them is essential. Motivation and engagement are some of the most commonly mentioned concepts in gamification, thus, a gamified application seems to have the necessary features to improve the motivation of students in the learning context of Ocean Literacy topics. The main aims of this work were: i) to understand through a systematic evaluation, how game elements affect the different motivation layers; and ii) to compare them in terms of enhancing the motivation to recycle among secondary school students (11-14 years). To measure students’ motivation, a pre-test and a post-test using a recycling situational motivation survey were administered in a classroom environment in Portugal and the UK. Findings show a trend regarding the effect of game elements mainly on the most autonomous forms of motivation. The different game elements, each one with different degrees of effects, were shown to have potential to increase motivation.

Recent findings indicate that different Endocrine Disrupting Chemicals (EDCs) interfere with lipid metabolic pathways in mammals and promote fat accumulation, a previously unknown site of action for these compounds. The antifoulant and environmental pollutant tributyltin (TBT), which causes imposex in gastropod snails, induces an “obesogenic” phenotype in mammals, through the activation of the nuclear receptors retinoid X receptor (RXR) and peroxisome proliferator-activated receptor gamma (PPARγ). In teleosts, the effects of TBT on the lipid metabolism are poorly understood, particularly following exposure to low, environmental concentrations. In this context, the present work shows that exposure of zebrafish to 10 and 50 ng/L of TBT (as Sn) from pre-hatch to 9 months of age alters the body weight, condition factor, hepatosomatic index and hepatic triglycerides in a gender and dose related manner. Furthermore, TBT modulated the transcription of key lipid regulating factors and enzymes involved in adipogenesis, lipogenesis, glucocorticoid metabolism, growth and development in the brain and liver of exposed fish, revealing sexual dimorphic effects in the latter. Overall, the present study shows that the model mammalian obesogen TBT interferes with triglyceride accumulation and the transcriptional regulation of lipid metabolism in zebrafish and indentifies the brain lipogenic transcription profile of fish as a new target of this compound.