Human iPSC derived disease model of MERTK-associated retinitis pigmentosa

Retinitis pigmentosa (RP) represents a genetically heterogeneous group of retinal dystrophies affecting mainly the rod photoreceptors and in some instances also the retinal pigment epithelium (RPE) cells of the retina. Clinical symptoms and disease progression leading to moderate to severe loss of vision are well established and despite significant progress in the identification of causative genes, the disease pathology remains unclear. Lack of this understanding has so far hindered development of effective therapies. Here we report successful generation of human induced pluripotent stem cells (iPSC) from skin fibroblasts of a patient harboring a novel Ser331Cysfs*5 mutation in the MERTK gene. The patient was diagnosed with an early onset and severe form of autosomal recessive RP (arRP). Upon differentiation of these iPSC towards RPE, patient-specific RPE cells exhibited defective phagocytosis, a characteristic phenotype of MERTK deficiency observed in human patients and animal models. Thus we have created a faithful cellular model of arRP incorporating the human genetic background which will allow us to investigate in detail the disease mechanism, explore screening of a variety of therapeutic compounds/reagents and design either combined cell and gene- based therapies or independent approaches.

Perceived emotional intelligence, general intelligence and early professional success: predictive and incremental validity

Although the study of factors affecting career success has shown connections between biographical and other aspects related to ability, knowledge and personality, few studies have examined the relationship be-tween emotional intelligence and professional success at the initial career stage. When these studies were carried out, the results showed significant relationships between the dimensions of emotional intelligence (emotional self-awareness, self-regulation, social awareness or social skills) and the level of professional competence. In this paper, we analyze the relationship between perceived emotional intelligence, measured by the Trait Meta-Mood Scale (TMMS-24) questionnaire, general intelligence assessed by the Cattell factor "g" test, scale 3, and extrinsic indicators of career success, in a sample of 130 graduates at the beginning of their careers. Results from hierarchical regression analysis indicate that emotional intelligence makes a specific contribution to the prediction of salary, after controlling the general intelligence effect. The perceived emotional intelligence dimensions of TMMS repair, TMMS attention and sex show a higher correlation and make a greater contribution to professional success than general intelligence. The implications of these results for the development of socio-emotional skills among University graduates are discussed.